Case Reports in Hematology The latest articles from Hindawi Publishing Corporation © 2017 , Hindawi Publishing Corporation . All rights reserved. Allogeneic Transplant in ELANE and MEFV Mutation Positive Severe Cyclic Neutropenia: Review of Prognostic Factors for Secondary Severe Events Wed, 18 Jan 2017 09:58:59 +0000 Objective and Importance. Cyclic neutropenia (CyN) is a rare autosomal dominant inherited disorder due to the mutation ELANE primarily affecting bone marrow stem cells and is characterized by recurrent neutropenia every 2 to 4 weeks. Symptoms vary from benign to severe, including death. Postulations on the cause of wide spectrum in symptom presentation include the possibility of other genetic mutations, such as MEFV. Recommended treatment for CyN is G-CSF to keep ANC higher to minimize risk of infection. Case. A 25-year-old male diagnosed with CyN, on G-CSF but worsening quality of life. Pretransplant investigations revealed ELANE mutation positive severe CyN along with familial Mediterranean fever (MEFV) mutation. Intervention. Bone marrow transplantation as treatment for dual mutation (ELANE and MEFV mutation) positive severe CyN. Conclusion. BMT may be considered as an alternative treatment for severe CyN in patients who are refractory to G-CSF. It is postulated that in our patient the combined mutations (CyN and MEFV) may have contributed to the severity of this individual’s symptoms. We suggest CyN patients who present with severe symptoms have evaluation with ELANE mutation testing, Periodic Fever Syndromes Panel, and routine marrow assessment with FISH, conventional cytogenetics, and morphological evaluation for MDS/AML. Onyemaechi N. Okolo, Emmanuel Katsanis, Seongseok Yun, Candace Y. Reveles, and Faiz Anwer Copyright © 2017 Onyemaechi N. Okolo et al. All rights reserved. Hodgkin Lymphoma and Castleman Disease: When One Blood Disease Can Hide Another Wed, 18 Jan 2017 09:39:24 +0000 We describe a rare case of Castleman disease associated de novo with Hodgkin lymphoma. The incidence of Castleman disease is rare; only a few studies have described it in de novo association with Hodgkin lymphoma. The patient described here complained of unique evolutionary axillary adenopathy. A positron-emission tomography/computed tomography scan revealed hypermetabolic activity in this area. Diagnosis was based on a total excision biopsy of the adenopathy. The patient underwent complete remission with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) chemotherapy for treating Hodgkin lymphoma after surgical excision of the unicentric Castleman disease lesion. L. Filliatre-Clement, H. Busby-Venner, C. Moulin, G. Roth-Guepin, and A. Perrot Copyright © 2017 L. Filliatre-Clement et al. All rights reserved. Angioimmunoblastic T-Cell Lymphoma: A Questionable Association with Follicular Dendritic Cell Sarcoma Wed, 18 Jan 2017 00:00:00 +0000 An elderly woman presented with generalized lymphadenopathy, several systemic symptoms, and splenomegaly. An inguinal lymph node excision revealed a compound picture. One aspect of the lymph node morphology, including cells with follicular T-helper cell phenotype, was most consistent with angioimmunoblastic T-cell lymphoma. The other component, revealing spindle cells forming whorls with immunostaining for CD21, CD23, and fascin, might be an integral part of this T-cell lymphoma. However, due to the often massive involvement of the nodal tissue by these follicular dendritic cells, these areas were questionably suggestive of involvement by follicular dendritic cell sarcoma. We raise herein the issue of the borderline area between advanced follicular dendritic cell expansion in angioimmunoblastic T-cell lymphoma and a massive follicular dendritic cell proliferation consistent with follicular dendritic cells sarcoma, in the absence of a genomic analysis. Daniel Benharroch, Miriam Zekzer, and Karen Nalbandyan Copyright © 2017 Daniel Benharroch et al. All rights reserved. Management of Venous Thromboembolism in Patients with Hereditary Antithrombin Deficiency and Pregnancy: Case Report and Review of the Literature Tue, 10 Jan 2017 12:26:35 +0000 Background. Hereditary antithrombin deficiency is a thrombogenic disorder associated with a 50–90% lifetime risk of venous thromboembolism (VTE), which is increased during pregnancy and the puerperium in these patients. We present a case of a woman with antithrombin (AT) deficiency who presented with a VTE despite therapeutic low molecular weight heparin (LMWH). Though the pregnancy was deemed unviable, further maternal complications were mitigated through the combined use of therapeutic anticoagulation and plasma-derived antithrombin concentrate infusions to normalize her functional antithrombin levels. Methods. A review of the literature was conducted for studies on prophylaxis and management of VTE in pregnant patients with hereditary AT deficiency. The search involved a number of electronic databases, using combinations of keywords as described in the text. Only English language studies between 1946 and 2015 were included. Conclusion. Antithrombin concentrate is indicated in pregnant women with hereditary AT deficiency who develop VTE despite being on therapeutic dose anticoagulation. Expert opinion suggests AT concentrate should be used concomitantly with therapeutic dose anticoagulation. However, further high-quality studies on the dose and duration of treatment in the postpartum period are required. Use of AT concentrate for prophylaxis is controversial and should be based on individual VTE risk stratification. Mohammad Refaei, Lydia Xing, Wendy Lim, Mark Crowther, and Kochawan Boonyawat Copyright © 2017 Mohammad Refaei et al. All rights reserved. Rapid Change in Mental Status in a Patient with Hypereosinophilia Tue, 10 Jan 2017 08:58:19 +0000 We present the case of a 48-year-old female with acute onset altered mental status, who was found to have eosinophilia, elevated troponin, and embolic strokes. Extensive testing for autoimmune, infectious, and coronary artery etiologies was unremarkable. After a cardiac MRI revealed focal myocardial hyperenhancement, the patient underwent an endomyocardial biopsy with findings consistent with eosinophilic myocarditis. The patient was diagnosed of idiopathic hypereosinophilic syndrome and started on prednisone and apixaban. Our case highlights the importance of considering hypereosinophilic syndrome when eosinophilia is associated with multisystem impairments, as tissue biopsy is usually required to diagnose this rare condition. Hanyin Wang and John K. Erban Copyright © 2017 Hanyin Wang and John K. Erban. All rights reserved. Radiation for MALT of the Submandibular Gland Mon, 09 Jan 2017 09:07:00 +0000 We are reporting a case of a 27-year-old woman with a history of swelling in the left submandibular region. This swelling was associated with a mass, and this was pathologically confirmed to be an extranodal marginal zone lymphoma (MALT). The patient underwent surgical excision and postoperative adjuvant radiation therapy. The patient tolerated treatments well and remains free of disease. Here, we describe the case and management described in the current literature. Juskaran Chadha, Marita S. Teng, Julie Teruya-feldstein, and Richard L. Bakst Copyright © 2017 Juskaran Chadha et al. All rights reserved. Gelatinous Marrow Transformation Associated with Imatinib: Case Report and Literature Review Wed, 04 Jan 2017 09:37:48 +0000 Gelatinous marrow transformation (GMT) is a rare condition observed in severe illness or malnutrition, in which the bone marrow contains amorphous “gelatinous” extracellular material, and histopathology demonstrates varied degrees of fat cell atrophy and loss of hematopoietic elements. An association of GMT with imatinib use in chronic myeloid leukemia (CML) has been reported recently. The objective of this study is to describe a case of GMT associated with imatinib use and review the existing similar cases in the literature to identify epidemiological patterns and potential imatinib-induced mechanisms leading to gelatinous conversion. E. Chang, G. Rivero, B. Jiang, S. Yellapragada, and P. Thiagarajan Copyright © 2017 E. Chang et al. All rights reserved. Secondary Hemochromatosis due to Chronic Oral Iron Supplementation Wed, 04 Jan 2017 06:22:50 +0000 Iron may accumulate in excess due to a mutation in the HFE gene that upregulates absorption or when it is ingested or infused at levels that exceed the body’s ability to clear it. Excess iron deposition in parenchymal tissue causes injury and ultimately organ dysfunction. Diabetes mellitus and hepatic cirrhosis due to pancreas and liver damage are just two examples of diseases that result from iron overload. Despite the rapid growth of information regarding iron metabolism and iron overload states, the most effective treatment is still serial phlebotomies. We present a patient who developed iron overload due to chronic ingestion of oral ferrous sulfate. This case illustrates the importance of querying geriatric patients regarding their use of nonprescription iron products without a medical indication. Ronald Lands and Emmanuel Isang Copyright © 2017 Ronald Lands and Emmanuel Isang. All rights reserved. Neurological Complications following Blood Transfusions in Sickle Cell Anemia Tue, 03 Jan 2017 08:31:49 +0000 In Sickle Cell Anemia (SCA) patient blood transfusions are an important part of treatment for stroke and its prevention. However, blood transfusions can also lead to complications such as Reversible Posterior Leukoencephalopathy Syndrome (RPLS). This brief report highlights two cases of SCA who developed such neurological complications after a blood transfusion. RLPS should be considered as the cause of neurologic finding in patients with SCA and hypertension following a blood transfusion. Hana Alharbi, Nayaab Khawar, Jolanta Kulpa, Anne Bellin, Simona Proteasa, and Revathy Sundaram Copyright © 2017 Hana Alharbi et al. All rights reserved. Spontaneous Remission in an Older Patient with Relapsed FLT3 ITD Mutant AML Thu, 29 Dec 2016 14:06:45 +0000 Spontaneous remission (SR) of acute myeloid leukemia (AML) is a very rare phenomenon. AML characterized by FLT3 internal tandem duplication (FLT3 ITD) is typically associated with an aggressive clinical course with rapid progression, relapse, and short overall survival in the absence of transplantation. We report here the first case of SR of FLT3 ITD mutant AML in the literature. Our patient was an elderly woman with relapsed NPM1 and FLT3 ITD mutant AML whose disease underwent SR for a brief duration without precipitating cause. We review the potential immune mechanisms underlying SR in AML and discuss the implications for novel immunotherapeutic approaches for FLT3 mutant AML. Pankit Vachhani, Jason H. Mendler, Andrew Evans, George Deeb, Petr Starostik, Paul K. Wallace, and Eunice S. Wang Copyright © 2016 Pankit Vachhani et al. All rights reserved. Hypercalcemia due to Primary Hepatic Lymphoma Sun, 25 Dec 2016 11:57:56 +0000 A 65-year-old female with a history of mixed connective tissue disease and pulmonary fibrosis on azathioprine, hydroxychloroquine, and prednisone (osteoporosis on teriparatide) presented with a 1-month history of hypercalcemia. After discontinuation of teriparatide, the patient’s hypercalcemia persisted. Further evaluation revealed primary hepatic lymphoma as the source of her hypercalcemia. Andrew Hsu, Michael Gagnier, Elizabeth Ryer, Mohammed Salhab, and Alan G. Rosmarin Copyright © 2016 Andrew Hsu et al. All rights reserved. Attempted Suicide by Massive Warfarin Ingestion Conservatively Managed Using Phytonadione Sun, 04 Dec 2016 14:26:47 +0000 Treatment strategies for acute toxicity following massive ingestion of warfarin are not well described in the literature. Warfarin is the primary oral anticoagulation agent used in the treatment of thromboembolic disease, and patients with acute toxicity are at risk for life-threatening hemorrhages. Treatment options include phytonadione (vitamin K1), fresh frozen plasma (FFP), and prothrombin complex concentrates (PCCs) used alone or in combination. FFP and PCC can be associated with volume complications, undesirable thromboembolic events, and increased costs. We describe the case of a 63-year-old female with acute warfarin toxicity following a massive ingestion of warfarin (420 mg–450 mg) in an attempt to commit suicide. Upon arrival to the emergency department, serial INR checks were initiated to help guide dosing strategy and later adjusted based on INR response to treatment using only phytonadione. Katherine L. March, Kruti S. Patel, and Jennifer D. Twilla Copyright © 2016 Katherine L. March et al. All rights reserved. Pseudothrombocytopenia due to Platelet Clumping: A Case Report and Brief Review of the Literature Sun, 04 Dec 2016 11:04:30 +0000 Platelet clumping is a common laboratory phenomenon that complicates or precludes reporting of platelet count. It is often, but not always, a phenomenon commonly caused by the anticoagulant EDTA. Herein, we discuss a case of a 14-year-old girl who was found to have platelet clumping and discuss the work-up she underwent to investigate her pseudothrombocytopenia. Geok Chin Tan, Melissa Stalling, Gretchen Dennis, Maria Nunez, and Samir B. Kahwash Copyright © 2016 Geok Chin Tan et al. 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.