Case Reports in Hematology The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Minimally Invasive Diagnosis of Secondary Intracranial Lymphoma Mon, 28 Nov 2016 13:36:42 +0000 Diffuse large B cell lymphomas (DLBCL) are an aggressive group of non-Hodgkin lymphoid malignancies which have diverse presentation and can have high mortality. Central nervous system relapse is rare but has poor survival. We present the diagnosis of primary mandibular DLBCL and a unique minimally invasive diagnosis of secondary intracranial recurrence. This case highlights the manifold radiological contributions to the diagnosis and management of lymphoma. A. P. McClement, G. M. Healy, C. E. Redmond, E. Stocker, G. Connaghan, S. J. Skehan, and R. P. Killeen Copyright © 2016 A. P. McClement et al. All rights reserved. Coexistence of Factor VII Deficiency and Hereditary Spastic Paraplegia in Two Siblings Sun, 27 Nov 2016 12:30:24 +0000 We present the case of two patients aged 12 years and 7 years who were referred to our hospital for factor VII deficiency inherited in an autosomal recessive pattern, who had suffered from previous multiple joint haemarthroses. They presented with fine motor symptoms and difficulty in walking. During physical examination we observed neurological symptoms (general hypotonia, muscular hypotrophy, exaggerated tendon reflexes, pes cavus, and spastic gait). Given that the symptoms were not justified by the deficiency of coagulation factor VII and on suspicion of hereditary spastic paraplegia (HSP), tests were carried out. Findings from the tests confirmed the diagnosis of HSP (axonal degeneration of the central motor pathway and pyramidal tracts), further complicated by mixed neuropathy. This disease was also inherited in an autosomal recessive pattern with no direct genetic association with factor VII deficiency. Neurological symptoms had gone unnoticed due to a history of multiple joint haemarthrosis; musculoskeletal examination led to a satisfactory differential diagnosis. Haematological prophylaxis was commenced with rFVIIa at 30 mcg/kg, three days per week. A rehabilitation programme was prescribed so that the patient could remain independent for as long as possible, based on orthosis, physiotherapy, and occupational therapy. Response to treatment is currently satisfactory and no new bleeding has presented. As far as we are aware, the coexistence of these two diseases (factor VII deficiency and HSP) has not been previously reported in the literature. Hortensia De la Corte-Rodriguez, E. Carlos Rodriguez-Merchan, M. Teresa Alvarez-Roman, and Ana L. Hernandez-Moreno Copyright © 2016 Hortensia De la Corte-Rodriguez et al. All rights reserved. Ischemic Monomeric Neuropathy in a Woman with Sickle Cell Anaemia Tue, 22 Nov 2016 14:05:05 +0000 Sickle cell disease is an inherited haemoglobinopathy that can affect multiple organs and systems. The most common neurological complication in sickle cell disease is stroke and silent cerebral infarcts. Peripheral nervous system involvement has been described but is exceedingly rare. Herein, we describe the case of a young woman who presented with acute flaccid paralysis and sensory loss of the left lower extremity in the context of a painful vasoocclusive crisis which resolved rapidly after receiving an emergency automated red cell exchange transfusion. Alexandra Agapidou, Laura Aiken, Lisa Linpower, and Dimitris A. Tsitsikas Copyright © 2016 Alexandra Agapidou et al. All rights reserved. Total Body Irradiation without Chemotherapy as Conditioning for an Allogeneic Hematopoietic Cell Transplantation for Adult Acute Myeloid Leukemia Sun, 13 Nov 2016 09:49:22 +0000 Current therapies for acute myeloid leukemia (AML), failing induction, are rarely effective. We report our experience in 4 patients with AML who received 16 Gy TBI prior to allogeneic hematopoietic cell transplantation (alloHCT), between June 2010 and May 2011. Patients were 20 to 55 years of age, 2 with relapsed disease and 2 with AML failing induction. An HLA-matched graft from related or unrelated donor was infused on day 0. All but one, who received a CD34+-selected graft, received methotrexate and tacrolimus +/− antithymocyte globulin, as GVHD prophylaxis. The other patient received tacrolimus alone. Neutrophil and platelet engraftment occurred at a median of 18 and 14 days, respectively. Patients were discharged at a median of 28 days. There were no unexpected toxicities in the first 30 days. One patient had cytomegalovirus (CMV) viremia and anorexia, at two months. One patient had grade 2 acute GVHD of the skin. One patient developed chronic GVHD of the eyes, mouth, skin, joints, and lung at 4 months. Two patients died from relapse of their leukemia at days 65 and 125. Two patients remain in remission beyond day 1500. 16 Gy TBI followed by an alloHCT for AML, failing induction, is feasible and tolerable. Sultan Altouri, Mitchell Sabloff, David Allan, Harry Atkins, Lothar Huebsch, Dawn Maze, Rajiv Samant, and Christopher Bredeson Copyright © 2016 Sultan Altouri et al. All rights reserved. Bortezomib, Ifosfamide, Carboplatin, and Etoposide in a Patient with HIV-Negative Relapsed Plasmablastic Lymphoma Sun, 13 Nov 2016 08:09:59 +0000 Plasmablastic lymphoma (PBL) is a rare subtype of diffuse large B cell lymphoma (DLBCL), often associated with HIV infection. We present a case of a 53-year-old HIV-negative man with untreated hepatitis C viral infection who presented with abdominal pain and lymphadenopathy. Lymph node and bone marrow biopsies were consistent with plasmablastic lymphoma. He had partial response (PR) to 6 cycles of EPOCH but disease progressed seven weeks later. Repeat biopsy was consistent with plasmablastic lymphoma. Three cycles of bortezomib, ifosfamide, carboplatin, and etoposide (B-ICE) chemotherapy resulted in a partial response (PR). Five months later, he presented with widespread lymphadenopathy and tumor lysis syndrome with circulating blasts. Flow cytometry revealed a different population of lymphoma cells, this time positive for CD5, CD19, CD20, and CD22, with dim expression of CD45 and CD38. The patient died on the first day of ESHAP chemotherapy. There are no treatment recommendations or standard of care for plasmablastic lymphoma. A literature search yielded 10 cases in which bortezomib was administered in either HIV-positive or HIV-negative PBL. Six reported a partial response, 3 reported a complete response, and 1 was a near-complete response. Bortezomib, in combination with chemotherapy, may be an effective treatment option in PBL as reported here. Mehmet Akce, Elaine Chang, Mohammad Haeri, Mike Perez, Christie J. Finch, Mark M. Udden, and Martha P. Mims Copyright © 2016 Mehmet Akce et al. All rights reserved. Fludarabine Treatment of Patient with Chronic Lymphocytic Leukemia Induces a Digital Ischemia Thu, 03 Nov 2016 13:14:15 +0000 We report a 63-year-old man with a history of chronic lymphocytic leukemia (CLL) who presented with asymmetrical Raynaud’s phenomenon of sudden onset which progressed to acral gangrene rapidly in a week. These symptoms began approximately one week after the fourth cycle of fludarabine and cyclophosphamide chemotherapy and were accompanied by pain, numbness, and cyanosis in the fingers of his right hand except the first finger. Fludarabine may play a role in acral vascular syndrome. The treatment with fludarabine in patients with evolving digital ischemia should be carried out with caution. Utku Erdem Soyaltin, Deniz Yuce Yildirim, Mustafa Yildirim, Mehmet Can Ugur, Ferhat Ekinci, Cengiz Ceylan, and Harun Akar Copyright © 2016 Utku Erdem Soyaltin et al. All rights reserved. Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy Wed, 02 Nov 2016 14:14:35 +0000 Protein losing enteropathy (PLE) refers to excessive intestinal protein loss, resulting in hypoalbuminemia. Underlying pathologies include conditions leading to either reduced intestinal barrier or lymphatic congestion. We describe the case of a patient with long-lasting diffuse abdominal problems and PLE. Repetitive endoscopies were normal with only minimal lymphangiectasia in biopsies. Further evaluations revealed an indolent marginal zone lymphoma with minor bone marrow infiltration. Monotherapy with rituximab decreased bone marrow infiltration of the lymphoma but did not relieve PLE. Additional treatments with steroids, octreotide, a diet devoid of long-chain fatty-acids, and parenteral nutrition did not prevent further clinical deterioration with marked weight loss (23 kg), further reduction in albumin concentrations (nadir 8 g/L), and a pronounced drop in performance status. Finally, immunochemotherapy with rituximab and bendamustine resulted in hematological remission and remarkable clinical improvement. 18 months after therapy the patient remains free of gastrointestinal complaints and has regained his body weight with normal albumin levels. We demonstrate a case of PLE secondary to indolent marginal zone lymphoma. No intestinal pathologies were detected, contrasting a severe and almost lethal clinical course. Immunochemotherapy relieved lymphoma and PLE, suggesting that a high suspicion of lymphoma is warranted in otherwise unexplained cases of PLE. Nadine Stanek, Peter Bauerfeind, Guido Herzog, Henriette Heinrich, Matthias Sauter, Daniela Lenggenhager, Cäcilia Reiner, Markus G. Manz, Jeroen S. Goede, and Benjamin Misselwitz Copyright © 2016 Nadine Stanek et al. All rights reserved. Bilateral Numb Chin Syndrome as the Initial Presentation of Burkitt’s Lymphoma/Leukemia: A Report of Two Cases and Review of the Literature Tue, 01 Nov 2016 09:08:02 +0000 Numb chin syndrome, also known as mental nerve neuropathy, is a rare sensory neuropathy characterized by paresthesia and hypoesthesia in the area supplied by the mental nerve and its branches. This syndrome may be the first symptom of underlying malignancy or the first sign of recurrence and metastasis in patients with preexisting cancer. In this article, we present two cases with bilateral numb chin syndrome as the first manifestation of Burkitt’s lymphoma/leukemia and review the relevant literature. Numb chin syndrome should be considered as a warning sign and raise the suspicion for an underlying malignancy. Bilateral involvement is especially hazardous and must not be underestimated. In fact, an astute neurologist and internist who realizes that chin numbness is a potentially significant symptom can then exclude serious underlying malignancies. Standard diagnostic protocol with different modalities of imaging based on the availability and experience of the radiology team should be mandatory. High index of suspicion should be practiced to avoid delay in diagnosis and progression of the underlying malignancy. Hussein Algahtani, Bader Shirah, Wafaa Bassuni, and Reem Adas Copyright © 2016 Hussein Algahtani et al. All rights reserved. Mantle Cell Hyperplasia of Peripheral Lymph Nodes as Initial Manifestation of Sickle Cell Disease Sun, 30 Oct 2016 11:48:36 +0000 Sickle cell disease (SCD) is a well known hemoglobinopathy with usual manifestations including anemia, hyperbilirubinemia, and vasoocclusive complications. Despite presence of mild splenomegaly in early phase of the disease, lymphadenopathy is not an often finding of SCD. We introduce an undiagnosed case of SCD who presented in third decade of his life with multiple cervical lymphadenopathies and mild splenomegaly persistent for about five years. Histopathologic examination of the resected lymph nodes showed expansion of the mantle cell layers of secondary follicles as well as several monomorphic mantle cell nodules. To rule out possibility of a malignant process involving lymph nodes, an immunohistochemical panel was ordered which was in favor of benign mantle cell hyperplasia. Immunoglobulin gene rearrangement study showed no clonal bands and confirmed benign nature of the process. Respecting mild abnormalities on Complete Blood Count, peripheral blood smear was reviewed revealing some typical sickle red blood cells as well as rare nucleated red blood cells. Solubility test for hemoglobin (HB) S was positive. Hemoglobin electrophoresis confirmed diagnosis of homozygous HbS disease. Ahmad Monabbati, Sadat Noori, Akbar Safaei, Mani Ramzi, Seyedsajjad Eghbali, and Ali Adib Copyright © 2016 Ahmad Monabbati et al. All rights reserved. Hip Replacement Surgery in 14-Year-Old Girl with Factor V Deficiency: Haemostatic Treatment and Thromboprophylaxis Sun, 30 Oct 2016 07:16:34 +0000 Factor V (FV) is a pivotal coagulation factor present in plasma and platelets. It plays an essential role in secondary haemostasis acting as a cofactor in the prothrombinase complex, catalysing the conversion of prothrombin to thrombin. There is little evidence on the management of mayor orthopaedic surgery in paediatric or adolescents subjects with this coagulopathy and almost no information about thromboprophylaxis in these situations. We report a case of a hip replacement in a 14-year-old girl with moderate FV deficiency (0.07 IU mL−1). As haemostatic replacement, inactivated fresh frozen plasma (FFP) was transfused at doses of 600 mL (15 mL kg−1, 45 kg weight) 2 hours before surgery and then sequential FFP infusions of 250 mL (7 mL kg−1) every 12 hours for 7 days. Plasma factor VIII, von Willebrand factor antigen, and von Willebrand ristocetin cofactor were monitored to avoid supranormal levels. Since the patient was sexually mature (Marshall and Tanner stage 5) with the hormone replacement therapy, she was immobilized and the surgery was considered as a high thrombotic risk. Thus, low molecular weight heparin was administered at doses of intermediate risk (Enoxaparin 20 mg daily, by weight) after finishing the daily infusion of plasma: 24 hours and during the 7 days after intervention. No tranexamic acid was used. No haemorrhagic or thrombotic adverse event was described. María Eva Mingot-Castellano, Josefina Pérez-Núñez, and Lourdes Baeza-Montañez Copyright © 2016 María Eva Mingot-Castellano et al. All rights reserved. Retreatment with Bendamustine-Bortezomib-Dexamethasone in a Patient with Relapsed/Refractory Multiple Myeloma Thu, 27 Oct 2016 14:24:39 +0000 The clinical management of relapsed/refractory multiple myeloma and the correct choice of the most suitable therapy in heavily pretreated and fragile patients are tough clinical issues for clinicians. In advanced phases of disease, the choice of available therapies becomes very poor, and the retreatment with previously adopted and effective therapy, although unpredictable, could be an effective option. In this report, we describe the clinical history of a patient, previously treated with 9 lines of therapy, refractory to bortezomib and IMIDs, for whom the retreatment with bendamustine resulted in a stable disease with good quality of life. Claudio Cerchione, Davide Nappi, Maria Di Perna, Irene Zacheo, Anna Emanuele Pareto, Marco Picardi, Lucio Catalano, and Fabrizio Pane Copyright © 2016 Claudio Cerchione et al. All rights reserved. Lymphoma Heterogeneity: Three Different Histological Pictures and One Unique Clone Thu, 27 Oct 2016 08:26:46 +0000 We report a patient who developed up to three different lymphomas with the same clonal IGH rearrangement. She was first diagnosed of splenic zone marginal lymphoma and relapsed for the first time with Hodgkin lymphoma histology and later with diffuse large B-cell lymphoma histology. Subsequent biopsies and analysis of clonally rearranged IGH genes helped to elucidate the clonal relationship between the three histologies and to confirm a common origin from the three tissue histologies. An integrated diagnosis should always be performed in order to achieve the most accurate diagnosis and be able to choose the best therapeutic options for our patients. Sara Alonso-Alvarez, Alba Redondo-Guijo, Óscar Blanco, Miguel Alcoceba, Ana Balanzategui, Juan C. Caballero, Julio Dávila, Marcos González, María D. Caballero, Alejandro Martín, and Ramón García-Sanz Copyright © 2016 Sara Alonso-Alvarez et al. All rights reserved. Reactivation of Pulmonary Tuberculosis following Treatment of Myelofibrosis with Ruxolitinib Sun, 23 Oct 2016 09:45:41 +0000 Ruxolitinib is widely in use for treatment of myeloproliferative disorders. It causes inhibition of the Janus kinase (JAK) signal transducer and activation of transcription (STAT) pathway, which plays a key role in the underlying pathophysiology of myeloproliferative diseases. We describe a case of reactivation pulmonary tuberculosis in a retired physician while on treatment with ruxolitinib. We also review the literature on opportunistic infections following use of ruxolitinib. Our case highlights the importance of screening for latent tuberculosis in patients from highly endemic areas prior to start of therapy with ruxolitinib. Maheen Z. Abidi, Javeria Haque, Parvathi Varma, Horatiu Olteanu, Guru Subramanian Guru Murthy, Binod Dhakal, and Parameswaran Hari Copyright © 2016 Maheen Z. Abidi et al. All rights reserved. Usefulness of Low-Dose Splenic Irradiation prior to Reduced-Intensity Conditioning Regimen for Hematopoietic Stem Cell Transplantation in Elderly Patients with Myelofibrosis Thu, 20 Oct 2016 12:56:17 +0000 The Janus kinase (JAK) 1 and 2 inhibitor, ruxolitinib, was recently approved in Japan and has been effective in many patients with myelofibrosis (MF). Although the inhibitor decreases splenomegaly and relieves MF-related symptoms, allogeneic hematopoietic cell transplantation (HCT) remains as the only curative therapy for MF. The presence of splenomegaly has been reported as a risk factor for graft failure, delayed engraftment, and poor survival. Here, we report two elderly MF patients with massive splenomegaly and a JAK2 V617F mutation. These patients underwent splenic irradiation to decrease splenomegaly prior to HCT with a reduced-intensity conditioning (RIC) regimen. Massive splenomegaly gradually decreased by 4 Gy splenic irradiation. The subsequent RIC regimen involved 4 Gy total body irradiation and fludarabine and intravenous busulfan. In both patients, engraftment failure did not occur, and complete remission was achieved. The splenomegaly decreased, and MF-related symptoms were resolved. Furthermore, the JAK2 V617F mutation disappeared, and fibrosis in the bone marrow regressed. We suggest that splenic irradiation prior to the RIC regimen for HCT in elderly MF patients with massive splenomegaly is safe. Furthermore, the HCT protocols with splenic irradiation should be considered for patients who have not shown clinical benefits to optimal medical management such as treatment with ruxolitinib. Etsuko Matsubara, Jun Yamanouchi, Riko Kitazawa, Taichi Azuma, Hiroshi Fujiwara, Takaaki Hato, and Masaki Yasukawa Copyright © 2016 Etsuko Matsubara et al. All rights reserved. Balancing Therapy with Thrombopoietin Receptor Agonists and Splenectomy in Refractory Immune Thrombocytopenic Purpura: A Case of Postsplenectomy Thrombocytosis Requiring Plateletpheresis Wed, 12 Oct 2016 09:10:12 +0000 Immune thrombocytopenic purpura (ITP) causes thrombocytopenia through the autoimmune destruction of platelets. Corticosteroids remain the first line of therapy, and traditionally splenectomy has been the second. While the availability of thrombopoietin receptor agonists (TPO-RAs) has expanded treatment options, there is little data for the ideal management of these agents in preparation for splenectomy. Thrombocytosis has been reported after splenectomy in patients treated with TPO-RA preoperatively, with one prior case requiring plateletpheresis for symptomatic thrombocytosis. We present a case report and review of the literature pertaining to this complication and provide recommendations for preventing postsplenectomy thrombocytosis in ITP patients on TPO-RAs. Jacquelyn Zimmerman, Kelly J. Norsworthy, and Robert Brodsky Copyright © 2016 Jacquelyn Zimmerman et al. All rights reserved. Uveitis and Myositis as Immune Complications in Chemorefractory NK/T-Cell Nasal-Type Lymphoma Successfully Treated with Allogeneic Stem-Cell Transplant Tue, 11 Oct 2016 13:03:28 +0000 NK/T-cell lymphomas are a group of clonal proliferations of NK- or, rarely, T-cell types and have peculiar clinicopathologic features. Most common site of involvement is the upper aerodigestive tract (nasal cavity, nasopharynx, paranasal sinuses, and palate). Association of autoimmune paraneoplastic disorders with NK/T-cell lymphomas is not well studied. Our patient was diagnosed with NK/T-cell lymphoma stage IV with skin involvement and treated frontline with CHOEP regimen. While he was under treatment, two immune complications presented: anterior uveitis of autoimmune origin refractory to steroids and myositis in lower limbs muscles. Autologous transplantation was rejected due to confirmed early relapse after first-line treatment, and the patient received second-line treatment according to the SMILE scheme, reaching complete response after four cycles. The patient underwent allogeneic transplantation and at the time of manuscript preparation is alive despite multiple complications. The disease should be suspected in patients with rhinitis or recurrent sinusitis, and early biopsy is recommended for all patients to avoid a delay in diagnosis. Our patient also presented symptoms of disease progression after first-line treatment, representing a paraneoplastic process, a very rare phenomenon in T-type lymphomas. This case is novel for the appearance of an inflammatory myositis, a histologically verified paraneoplastic phenomenon that responded to treatment for lymphoma. Maria José Gómez-Crespo, Aránzazu García-Raso, Jose Luis López-Lorenzo, Teresa Villaescusa, María Rodríguez-Pinilla, José Fortes, Cristina Serrano, Salma Machan, Pilar Llamas, and Raúl Córdoba Copyright © 2016 Maria José Gómez-Crespo et al. All rights reserved. Massive Bleeding as the First Clinical Manifestation of Metastatic Prostate Cancer due to Disseminated Intravascular Coagulation with Enhanced Fibrinolysis Mon, 10 Oct 2016 11:31:56 +0000 Disseminated intravascular coagulation (DIC) is the most frequent coagulation disorder associated with metastatic prostate adenocarcinoma. However, DIC with enhanced fibrinolysis as an initial presentation of prostate cancer is extremely rare. The appropriate treatment to control bleeding in these situations is challenging, controversial, and based on isolated case reports in the literature. A 66-year-old male presented at the emergency department with acute severe spontaneous ecchymoses localized to the limbs, laterocervical hematoma, and hemothorax. Prostate specific antigen level was 385 μg/L, bone scintigraphy revealed multiple bone metastases, and prostate biopsy confirmed adenocarcinoma (Gleason 9; 4 + 5). Laboratory investigation showed a pattern of enhanced fibrinolysis rather than the more common intravascular coagulation mechanism. Epsilon aminocaproic acid in monotherapy was initiated with a clear and rapid control of bleeding manifestations. This rare case of massive bleeding due to DIC with enhanced fibrinolysis as the first manifestation of prostate cancer suggests that in selected cases where the acute bleeding dyscrasia is clearly associated with a dominant fibrinolysis mechanism it is possible to use an approach of monotherapy with antifibrinolytics. Mónica Palma Anselmo, Gustavo Nobre de Jesus, João Madeira Lopes, Rui M. M. Victorino, and João Meneses Santos Copyright © 2016 Mónica Palma Anselmo et al. All rights reserved. Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities Mon, 10 Oct 2016 07:49:12 +0000 Rendu-Osler-Weber syndrome, or hereditary hemorrhagic teleangiectasia (HHT), is a rare autosomal dominant vascular disorder, characterized by multiple mucocutaneous teleangiectases with recurrent nasal and gastrointestinal bleedings and/or solid-organ arteriovenous shunts. We describe the first case to our knowledge of pancytopenia in a 53-year-old patient, with a known history of HHT and recurrent nasal and gastrointestinal bleedings, who was found to have a major splenic artery aneurysm and other uncommon vascular abnormalities. In the absence of other evident causes of pancytopenia, hypersplenism was diagnosed. The patient underwent coil embolization of the splenic artery aneurysm, followed by rapid and sustained increase of white blood cell and platelet count. Splenic artery aneurysms are extremely uncommon in HHT as only anecdotal cases have been reported to date. However, we believe that the aneurysm critically contributed to the progression of splenomegaly and the development of pancytopenia. Nicolò Binello, Antonio Gasbarrini, and Eleonora Gaetani Copyright © 2016 Nicolò Binello et al. All rights reserved. Hemophagocytic Lymphohistiocytosis in a Patient with Classical Hodgkin Lymphoma Mon, 10 Oct 2016 06:20:43 +0000 Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome that can be associated with inherited genetic mutations, malignancy, autoimmune disorders, and viral infections. Though the pathogenesis is not fully known, HLH is understood to be a reactive process in the setting of uncontrolled activation of macrophages, CD8+ cytotoxic lymphocytes, and other immune cells. Hallmark clinicopathological features of HLH include fevers, cytopenias, hepatosplenomegaly, and hemophagocytosis in the bone marrow. Case Presentation. A previously healthy 28-year-old Caucasian male presented with a one-month history of persistent fever, night sweats, and unintentional weight loss. He was diagnosed with classical Hodgkin Lymphoma (HL) by core-needle biopsy of an axillary lymph node. Both bone marrow involvement by HL and hemophagocytosis were seen on subsequent bone marrow biopsy. Other findings included pancytopenia, splenomegaly, and elevated serum ferritin. Extensive work-up for autoimmune and infectious etiologies was unremarkable. The patient had a complete response after chemotherapy with Adriamycin, bleomycin, vincristine, and dacarbazine. Conclusion. This report documents the exceedingly uncommon association between HLH and HL. HLH is a hyperinflammatory syndrome with high mortality, so it is imperative to identify and treat the underlying cause for secondary HLH. Malignancy-associated HLH should be considered in the differential diagnosis for cancer patients who present with fever, cytopenias, and splenomegaly. G. Hyun, K. J. Robbins, N. Wilgus, L. Grosso, and S. D. Goyal Copyright © 2016 G. Hyun et al. All rights reserved. Chronic Disseminated Candidiasis Complicated by Immune Reconstitution Inflammatory Syndrome in Child with Acute Lymphoblastic Leukemia Sun, 09 Oct 2016 09:53:38 +0000 Hepatosplenic candidiasis also known as chronic disseminated candidiasis is a rare manifestation of invasive fungal infection typically observed in patients with acute leukemia in prolonged, deep neutropenia. Immune reconstitution inflammatory syndrome (IRIS) is an inflammatory disorder triggered by rapid resolution of neutropenia. Diagnosis and treatment of IRIS are still challenging due to a variety of clinical symptoms, lack of certain diagnostic criteria, and no standards of treatment. The diagnosis of IRIS is even more difficult in patients with hematological malignancies complicated by “probable” invasive fungal infection, when fungal pathogen is still uncertain. We report a case of probable hepatic candidiasis in 4-year-old boy with acute lymphoblastic leukemia. Despite proper antifungal therapy, there was no clinical and radiological improvement, so diagnosis of Candida-related IRIS was made and corticosteroid therapy was added to antifungal treatment achieving prompt resolution of infection symptoms. Olga Zając-Spychała, Bogna Ukielska, Katarzyna Jończyk-Potoczna, Benigna Konatkowska, and Jacek Wachowiak Copyright © 2016 Olga Zając-Spychała et al. All rights reserved. TAFRO Syndrome Associated with EBV and Successful Triple Therapy Treatment: Case Report and Review of the Literature Thu, 29 Sep 2016 16:57:38 +0000 TAFRO syndrome is a rare constellation of symptoms: thrombocytopenia, anasarca, reticulin fibrosis of the bone marrow, renal dysfunction, and organomegaly. Its pathogenesis involves an excessive and inappropriate cytokine storm, most notably from IL-6, causing multiorgan failure; however, its etiology is undetermined. Starting in 2012, TAFRO syndrome was first identified in Japan as an atypical variant of Castleman’s disease. Previous reports include various different treatment protocols with inconsistent survival outcomes. Here we report the first known American, EBV positive but HIV and HHV-8 negative, male with TAFRO syndrome. He was successfully treated with an unusual three-drug regimen including tocilizumab, etoposide, and rituximab. We review the literature of TAFRO syndrome, discuss its possible viral etiology, and propose an original treatment regimen. Malorie Simons, Emmanuel Apor, James N. Butera, and Diana O. Treaba Copyright © 2016 Malorie Simons et al. All rights reserved. A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure Thu, 29 Sep 2016 12:55:03 +0000 Intravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma. It usually presents with CNS and skin involvement. A 65-year-old Caucasian female presented with fevers and chills of 3-4 months’ duration. Bone marrow biopsy done 3 months prior showed no significant myelodysplasia or lymphoid aggregates. The patient later died due to multiorgan failure. A bone marrow biopsy showed 20–30% CD5+ B cells consistent with infiltrative large B-cell lymphoma. An autopsy performed revealed diffuse intravascular invasion by lymphoma cells. Multiorgan involvement by intravascular B-cell lymphoma is very rare. Based on our literature review and to the best of our knowledge, there are only 5 case reports describing the presentation of this lymphoma with multiorgan failure. The immunophenotypic studies performed revealed that our patient had de novo CD5+ intravascular large B-cell lymphoma which is known to be aggressive with very poor prognosis. Although it is an extremely rare lymphoma, it should be considered as a potential cause of multiorgan failure when no other cause has been identified. A prompt tissue diagnosis and high-dose chemotherapy followed by ASCT can sometimes achieve remission. Daulath Singh, Devika Kapuria, Suparna Nanua, and Rakesh Gaur Copyright © 2016 Daulath Singh et al. All rights reserved. CD7 Positive Diffuse Large B-Cell Lymphoma Arising in a Background of Follicular Lymphoma: A Case Report and Review of the Literature Wed, 28 Sep 2016 11:21:06 +0000 Diffuse large B-cell lymphoma (DLBCL) is a neoplasm of large B-lymphocytes with a diffuse growth pattern. The neoplastic cells express B-cell markers such as CD20 and PAX-5 and there may be coexpression of BCL-2, BCL-6, CD10, and MUM-1. With the exception of CD5, other T-cell markers are not commonly expressed in this neoplasm. Here, we describe the first reported case of a DLBCL with abnormal expression CD7 arising in a background of follicular lymphoma in an 81-year-old male who presented with a nontender left axillary mass. Additionally, no other T-cell antigens were expressed in this B-cell lymphoma. Expression of CD7 in DLBCL is exceptionally rare and its prognostic significance is unknown. Here, we describe this rare case with review of literature of known DLBCLs with expression of T-cell antigens. Elham Vali Betts and Hooman H. Rashidi Copyright © 2016 Elham Vali Betts and Hooman H. Rashidi. All rights reserved. Myeloproliferative Neoplasm or Reactive Process? A Rare Case of Acute Myeloid Leukemia and Transient Posttreatment Megakaryocytic Hyperplasia with JAK-2 Mutation Mon, 26 Sep 2016 12:07:39 +0000 Myeloproliferative neoplasms (MPNs) are hematopoietic malignancies characterized by unchecked proliferation of differentiated myeloid cells. The most common BCR-ABL1-negative MPNs are polycythemia vera, essential thrombocythemia, and primary myelofibrosis. The discovery of JAK2 V617F mutation has improved our understanding of the molecular basis of MPN. The high frequency of JAK2 mutation in MPN makes JAK2 mutation testing an essential diagnostic tool and potential therapeutic target for MPN. Here, we present a rare case of a 34-year-old patient who was initially diagnosed with acute myeloid leukemia (AML) with mutated NPM1. After chemotherapy treatment followed by granulocyte colony stimulating factor administration, the patient achieved complete remission of AML. However, the bone marrow showed hypercellularity with granulocytic hyperplasia, markedly increased atypical megakaryocytes (50.2/HPF) with focal clustering, and reticulin fibrosis (3/4). JAK2 V617F mutation was also detected. Considering the possibility of AML transformed from a previous undiagnosed MPN, patient underwent peripheral blood allogenic stem cell transplant. This case illustrates the diagnostic challenges of firmly establishing a diagnosis between similar, but distinct, disease entities and an accurate clinicopathological differentiation is crucial. Steven Wang, Jie Yan, Guangde Zhou, Rebecca Heintzelman, and J. Steve Hou Copyright © 2016 Steven Wang et al. All rights reserved. Sjögren’s Syndrome Complicated by Myeloid/Natural Killer Cell Precursor Acute Leukemia: Case Report and Review of the Literature Sun, 25 Sep 2016 09:41:31 +0000 We report a case of Sjögren’s syndrome (SS) complicated by myeloid/natural killer (NK) cell precursor acute leukemia (M/NKPAL). A 75-year-old woman with a previous SS history for 2 years was routinely treated. Peripheral blood progenitor cells were increased, and subsequent bone marrow cell morphology examination showed the presence of acute myeloid leukemia type M4. However, flow cytometry analysis revealed that CD7/CD56/CD33/CD34/HLA-DR/cCD3 were all positive and myeloperoxidase- (MPO-) specific staining, other T cells, NK cells, and myeloid markers were all negative. Clonal T-cell receptor (TCR)β/TCRγ/TCRδ gene rearrangements and Epstein-Barr virus (EBV) were negative. The diagnosis of M/NKPAL was therefore confirmed. Unfortunately, this patient did not receive chemotherapy and later died of acute left heart failure and respiratory failure. SS complication with M/NKPAL is relatively rare. Combined with the relevant literatures, our study offers new insights into the clinical characteristics, pathological features, possible pathogenesis, and differential diagnosis of this rare disease. Hao Feng, Jianlin Qiao, Ningning Ding, Wei Chen, Kunming Qi, Xiuying Pan, Jiang Cao, and Kailin Xu Copyright © 2016 Hao Feng et al. All rights reserved. A Case Study of Deep Vein Thrombosis of the Right Internal Jugular Vein in a Healthy 21-Year-Old Male Tue, 20 Sep 2016 12:08:46 +0000 We are reporting a case of a healthy 21-year-old male, with no significant past medical history, who was found to have an incidental nonocclusive deep vein thrombosis in the right internal jugular vein detected on a head MRI previously ordered for work-up of headaches. A follow-up upper extremity venous Doppler ultrasound confirmed the presence of a partially occlusive deep vein thrombosis in the right jugular vein. The case presented is unique for the reason that the patient is young and has no prior risk factor, personal or familial, for venous thrombosis except for associated polycythemia on clinical presentation. Javier Corral and Geri Villanueva Copyright © 2016 Javier Corral and Geri Villanueva. All rights reserved. Primary Diffuse Large B-Cell Lymphoma Localized to the Lacrimal Sac: A Case Presentation and Review of the Literature Thu, 08 Sep 2016 18:00:52 +0000 We report a rare case of diffuse large B-cell lymphoma (DLBCL) of the lacrimal sac in a 50-year-old male. The incidence of primary ocular lymphoma is low and it is considered a rare disease. Moreover, reports of ocular DLBCL are uncommon and the disease remains poorly characterized. Our patient presented for management of osteomyelitis and was incidentally found to have a painless swelling and cyst around his right eye. A PET/CT scan revealed hypermetabolic activity within the lacrimal sac and a subsequent excisional biopsy of the mass yielded histopathology consistent with DLBCL. Consequently, the patient underwent treatment with R-CHOP therapy. The patient responded well to chemotherapy with a substantial shrinkage in tumor burden and the disease remained localized. Herein, we present a rare case of primary ocular lymphoma, highlight the importance of early diagnosis, and review current treatment modalities. Kevin Zarrabi, Ved Desai, Brandom Yim, and Theodore G. Gabig Copyright © 2016 Kevin Zarrabi et al. All rights reserved. Immune Thrombocytopenia in a Child with T Cell Lymphoblastic Lymphoma Wed, 07 Sep 2016 12:55:20 +0000 We describe the case of a 13-year-old boy who presented with persistent thrombocytopenia during maintenance chemotherapy with mercaptopurine and methotrexate for T cell lymphoblastic lymphoma. He was diagnosed with immune thrombocytopenia (ITP) after thorough investigations for the relapse of lymphoma and was successfully treated with immunoglobulin and steroids. ITP is known to be associated with chronic lymphocytic leukemia, Hodgkin lymphoma, and various types of non-Hodgkin lymphoma but rarely with T cell non-Hodgkin lymphoma or in children. Diagnosis of ITP with lymphoma is challenging due to the many factors affecting platelet counts, and ITP often complicates the diagnosis or treatment course of lymphoma. The underlying mechanism of ITP with NHL is still unclear. Drug-induced immunomodulation with a reduction of regulatory T cells might have contributed to the development of ITP in our case. Kayo Tokeji, Sachi Sakaguchi, Tomoko Kurimoto, Junya Fujimura, and Toshiaki Shimizu Copyright © 2016 Kayo Tokeji et al. All rights reserved. Acute Myeloid Leukemia Relapse Presenting as Complete Monocular Vision Loss due to Optic Nerve Involvement Wed, 07 Sep 2016 11:32:12 +0000 Acute myeloid leukemia (AML) involvement of the central nervous system is relatively rare, and detection of leptomeningeal disease typically occurs only after a patient presents with neurological symptoms. The case herein describes a 48-year-old man with relapsed/refractory AML of the mixed lineage leukemia rearrangement subtype, who presents with monocular vision loss due to leukemic eye infiltration. MRI revealed right optic nerve sheath enhancement and restricted diffusion concerning for nerve ischemia and infarct from hypercellularity. Cerebrospinal fluid (CSF) analysis showed a total WBC count of 81/mcl with 96% AML blasts. The onset and progression of visual loss were in concordance with rise in peripheral blood blast count. A low threshold for diagnosis of CSF involvement should be maintained in patients with hyperleukocytosis and high-risk cytogenetics so that prompt treatment with whole brain radiation and intrathecal chemotherapy can be delivered. This case suggests that the eye, as an immunoprivileged site, may serve as a sanctuary from which leukemic cells can resurge and contribute to relapsed disease in patients with high-risk cytogenetics. Shyam A. Patel Copyright © 2016 Shyam A. Patel. All rights reserved. A 26-Year-Old Female with Systemic Mastocytosis with Associated Myeloid Neoplasm with Eosinophilia and Abnormalities of PDGFRB, t(4;5)(q21;q33) Thu, 25 Aug 2016 13:33:43 +0000 Various translocations involving the PDGFRB gene are identified in myeloid neoplasms. However, the PRKG2/PDGFRB fusion gene associated with t(4;5)(q21;q33) has previously been reported in only 3 patients. We present the case of a 26-year-old woman with microcytic anemia, basophilia, thrombocytosis, and massive splenomegaly, who was found to have systemic mastocytosis and associated clonal hematological non-mast cell lineage disease (SM-AHNMD), with myeloid neoplasm with PRKG2/PDGFRB rearrangement. Initial findings included basophilia (37%, 4.1‚ÄČk/L), hypercellular marrow with eosinophilia, and increased and atypical megakaryocytes, suggestive of myeloproliferative neoplasm. Additional studies revealed large clusters of CD25 positive mast cells, fulfilling the criteria for the diagnosis of systemic mastocytosis. Consistent with prior reports of this translocation, our patient has responded well to imatinib. This case, in conjunction with others in the literature, suggests a possible connection between t(4;5)(q21;q33) PRKG2/PDGFRB and systemic mastocytosis and highlights their favorable response to imatinib. Laura E. Brown, Da Zhang, Diane L. Persons, Abdulraheem Yacoub, Shivani Ponnala, and Wei Cui Copyright © 2016 Laura E. Brown et al. All rights reserved.