Case Reports in Hematology The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . 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. Long-Term Treatment with Romidepsin in Patients with Peripheral T-Cell Lymphoma Thu, 25 Aug 2016 13:07:30 +0000 Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of aggressive non-Hodgkin lymphomas. Angioimmunoblastic T-cell lymphoma (AITL) is a common subtype of PTCL, and patients with AITL typically have a poor prognosis with limited treatment options. Clinical studies have demonstrated the activity of romidepsin, a structurally unique, potent, bicyclic class 1 selective histone deacetylase inhibitor, in patients with relapsed or refractory AITL. In the case presented herein, we describe a patient treated with single-agent romidepsin at first diagnosis of AITL, resulting in complete remission for over 2 years and leading to the use of maintenance dosing. The patient eventually underwent a successful autologous stem cell transplant. This case illustrates the successful use of romidepsin for the long-term treatment of a patient with AITL in a clinical setting. Maintenance dosing may be an option for patients who have an extended response to romidepsin in order to optimize outcomes and to prolong time to the next subsequent line of therapy. In our case, the patient was able to remain in complete remission for more than 1 year while receiving maintenance dosing of romidepsin. Claudius Irlé and Jonathan Weintraub Copyright © 2016 Claudius Irlé and Jonathan Weintraub. All rights reserved. A Case of p63 Positive Diffuse Large B Cell Lymphoma of the Bladder Thu, 25 Aug 2016 13:01:49 +0000 Diffuse large B cell lymphoma (DLBCL), currently the most common type of non-Hodgkin lymphoma (NHL), is an aggressive B cell neoplasm that typically presents in older adults as a rapidly enlarging mass. The enlarging mass typically represents a lymph node, although extranodal disease can occur in a significant percentage (40%) of cases. The most common extranodal sites of involvement include the gastrointestinal tract and skin; primary bladder lymphoma represents only 0.2% of extranodal non-Hodgkin lymphomas. We report a case of diffuse large B cell lymphoma occurring in the bladder of an 83-year-old gentleman with an initial presentation of hematuria. This neoplasm displayed large, atypical cells with vesicular chromatin and prominent nucleoli that involved the bladder mucosa with invasion into muscularis propria, prostate, and urethra. Positive staining for p63 initially raised suspicion for poorly differentiated urothelial carcinoma; however, lack of staining for pancytokeratin and positive staining for LCA, CD20, CD79a, and PAX-5 confirmed the diagnosis of diffuse large B cell lymphoma. Though it does not occur in all cases, p63 can be positive in a significant percentage of cases of DLBCL; therefore, a diagnosis of lymphoma remains an important entity on the differential diagnosis of aggressive and particularly poorly differentiated neoplasms. Chelsey D. Deel, Carol Jones, and Teresa Scordino Copyright © 2016 Chelsey D. Deel et al. All rights reserved. Bacteremia due to Leuconostoc pseudomesenteroides in a Patient with Acute Lymphoblastic Leukemia: Case Report and Review of the Literature Mon, 22 Aug 2016 14:23:54 +0000 Leuconostoc species are vancomycin-resistant Gram-positive cocci. Infections due to Leuconostoc species have been reported in various immunocompromised patients, but little is known about such infection in patients with hematologic malignancies. We report a case of Leuconostoc infection in a 44-year-old woman with acute lymphoblastic leukemia. The patient developed a high fever despite antimicrobial therapy with doripenem after induction chemotherapy. After an isolate from blood cultures was identified as L. pseudomesenteroides, we changed the antibiotics to piperacillin-tazobactam and gentamicin, after which the patient recovered from the infection. Physicians should be aware of Leuconostoc species as causative pathogen if they encounter Gram-positive cocci bacteremia resistant to standard antibiotics such as vancomycin and teicoplanin, especially in patients with hematologic malignancies. Kazuko Ino, Kazunori Nakase, Kei Suzuki, Akiko Nakamura, Atsushi Fujieda, and Naoyuki Katayama Copyright © 2016 Kazuko Ino et al. All rights reserved. Acute Monocytic Leukemia Masquerading Behçet’s Disease-Like Illness at Onset in an Elderly Female Tue, 16 Aug 2016 16:07:00 +0000 A previously healthy 74-year-old Japanese female was hospitalized with fever and high C-reactive protein. She developed palatal herpangina-like aphthous ulcers, localized intestinal wall thickening, terminal ileum ulcers, and an erythematous acneiform rash; thus Behçet’s disease-like illness was suspected. Significant peripheral blood acute monocytosis developed during her hospitalization and acute monocytic leukemia (FAB M5b) with normal karyotype was diagnosed. By immunostaining, the infiltrating cells in the skin and the terminal ileum were identified as monocytic leukemic cells. This case exhibited a unique initial presentation of Behçet’s disease-like illness associated with acute monocytic leukemia. Shigeru Koba, Toshio Sekioka, Sorou Takeda, Aya Miyagawa-Hayashino, Keisuke Nishimura, and Shinsaku Imashuku Copyright © 2016 Shigeru Koba et al. All rights reserved. Simultaneous Manifestation of Chronic Myelomonocytic Leukemia and Multiple Myeloma during Treatment by Prednisolone and Eltrombopag for Immune-Mediated Thrombocytopenic Purpura Sun, 14 Aug 2016 06:10:54 +0000 An 80-year-old man was admitted to our hospital because of severe thrombocytopenia. He was diagnosed with idiopathic thrombocytopenia, and prednisolone together with eltrombopag was started, leading to significant improvement of platelet counts. Four years later, there was a prominent increase of peripheral blood monocytes, which was accompanied by recurrence of thrombocytopenia. Bone marrow aspirates and serum electrophoresis revealed coexistence of chronic myelomonocytic leukemia (CMML) and multiple myeloma (MM). The patient received lenalidomide plus dexamethasone therapy but died due to exacerbation of the disorder. It was supposed that thrombocytopenia was secondarily caused by CMML and MM developed at a later period. Masao Hagihara, Morihiro Inoue, Kenichiro Kodama, Tomoyuki Uchida, and Jian Hua Copyright © 2016 Masao Hagihara et al. All rights reserved. Case Report of Diffuse Large B Cell Lymphoma of Uterine Cervix Treated at a Semiurban Cancer Centre in North India Thu, 11 Aug 2016 16:14:14 +0000 Lymphoma of the uterine cervix is very rare. We report a case of diffuse large B cell lymphoma (DLBCL) involving the uterine cervix treated at a newly commissioned semiurban cancer centre in north India in 2015. Data for this study was obtained from the hospital electronic medical records and the patient’s case file. We also reviewed published case reports of uterine and cervical lymphoma involving forty-one patients. We treated a case of stage IV DLBCL cervix with six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) and intrathecal methotrexate followed by consolidation with radiotherapy. The patient showed complete response to chemotherapy. We conclude that, in advanced stage lymphoma involving uterus and cervix, combination of chemotherapy and radiotherapy is effective in short term. Vibhor Sharma, Tapas Dora, Mehul Patel, Sankalp Sancheti, and Epari Sridhar Copyright © 2016 Vibhor Sharma et al. All rights reserved. A Rare Cause of Hemophagocytic Lymphohistiocytosis: Fusobacterium Infection—A Case Report and Review of the Literature Wed, 03 Aug 2016 13:51:09 +0000 Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome characterized by excessive activation of the immune system. Bacterial infections are very rare precipitants of this disease. A 19-year-old gentleman presented with headache, fatigue, and malaise. He was found to be hypotensive, tachycardic, and febrile. Broad spectrum antibiotics were initiated, and a lumbar puncture ruled out meningitis. Patient progressively developed shock that required use of vasopressors, as well as renal and respiratory failure. Blood cultures grew Fusobacterium necrophorum. Given continued fevers despite appropriate antimicrobials, a bone marrow biopsy was performed revealing increased histiocytes with hemophagocytosis. Dexamethasone was added with dramatic clinical improvement. Our case highlights Fusobacterium as a rare precipitant of HLH and proves that a high index of clinical suspicion is crucial for early diagnosis of HLH, allowing for prompt initiation of HLH-specific immunosuppressive therapy that can be life-saving. Ghulam Rehman Mohyuddin and Heather J. Male Copyright © 2016 Ghulam Rehman Mohyuddin and Heather J. Male. All rights reserved. Steroid Refractory Autoimmune Haemolytic Anaemia Secondary to Sarcoidosis Successfully Treated with Rituximab and Mycophenolate Mofetil Tue, 02 Aug 2016 05:56:56 +0000 Autoimmune haemolytic anaemia is not a well-recognised complication of sarcoidosis. We describe the case of a 30-year-old female who presented with acute warm haemolytic anaemia and widespread lymphadenopathy. Sarcoidosis was diagnosed on lymph node biopsy and further investigation. The haemolytic anaemia responded only to a high dose of steroids. Evidence regarding treatment of steroid refractory autoimmune haemolysis secondary to sarcoidosis is lacking. Based on the emergent evidence that both disorders share common immunopathogenic mechanisms involving Th1 and Th17 lymphocytes, our patient was given rituximab and mycophenolate mofetil to successfully suppress the haemolysis and sarcoid activity. Sarah Green, Erica Partridge, Edore Idedevbo, and Anton Borg Copyright © 2016 Sarah Green et al. All rights reserved. Pernicious Anemia with Autoimmune Hemolytic Anemia: A Case Report and Literature Review Sun, 31 Jul 2016 07:22:16 +0000 Pernicious anemia is a common cause of vitamin B12 deficiency. Here, we discuss a case of a young woman who presented with severe anemia along with a history of iron deficiency anemia. After a review of her clinical presentation and laboratory data, we identified an autoimmune hemolytic anemia and a concomitant pernicious anemia. The concurrence of both these hematological diagnoses in a patient is rare. Sri Lakshmi Hyndavi Yeruva, Raj Pal Manchandani, and Patricia Oneal Copyright © 2016 Sri Lakshmi Hyndavi Yeruva et al. All rights reserved. Extranodal NK/T Cell Lymphoma Causing Cardiorespiratory Failure Sun, 17 Jul 2016 06:30:00 +0000 Extranodal NK/T cell lymphoma is an uncommon malignancy usually involving the sinonasal area. We report an unusual case of extranodal NK/T cell lymphoma diagnosed in a 62-year-old Caucasian male who died of progressive cardiorespiratory failure but had no clinically detectable upper respiratory system lesions. The initial diagnosis was made cytologically on a sample of pericardial fluid that contained neoplastic lymphoid cells. These cells were positive for CD2, cytoplasmic CD3, and Epstein-Barr virus and negative for CD56. The diagnosis was confirmed at the autopsy, which disclosed lymphoma infiltrates in the myocardium, lungs, stomach, and pancreas. The death was caused by heart and lung failure due to uncontrollable arrhythmia and respiratory insufficiency due to the lymphoma infiltrates. To the best of our knowledge, this is the first case of extranodal NK/T cell lymphoma presenting with cardiopulmonary failure. Yiting Li and Ivan Damjanov Copyright © 2016 Yiting Li and Ivan Damjanov. All rights reserved.