Case Reports in Hematology The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . All rights reserved. A Case of a Laryngeal MALT Lymphoma in a Patient with a History of Gastric MALT Sun, 18 Jan 2015 07:28:50 +0000 We are reporting a case of a 62-year-old African American woman with a history of gastric MALT lymphoma successfully treated with radiation who presented with a laryngeal MALT lymphoma 4 years after her original diagnosis. She received definitive radiation with a complete response. The case presented is unique for the rare presentation of a MALT lymphoma in the larynx, especially in light of the patient’s previously treated gastric MALT lymphoma years ago. Mark Ashamalla, Marita S. Teng, Joshua Brody, Elizabeth Demicco, Rahul Parikh, Kavita Dharmarajan, and Richard L. Bakst Copyright © 2015 Mark Ashamalla et al. All rights reserved. Human Herpesvirus-6 Pneumonitis around the Engraftment of Cord Blood Transplantation following Foscarnet Prophylaxis in a Patient with Acute Leukemia Sun, 11 Jan 2015 12:48:43 +0000 Human herpesvirus-6 (HHV-6) reactivation is sometimes observed in immunocompromised patients, especially after allogeneic stem cell transplantation. The complications of HHV-6 reactivation in this setting are mainly recognized as HHV-6 encephalitis. We herein report the case of a patient who developed HHV-6 pneumonitis after cord blood transplantation (CBT). A 35-year-old male underwent CBT for T-cell/myeloid mixed phenotype acute leukemia and achieved neutrophil engraftment on day 31. He had received foscarnet as prophylaxis for HHV-6 reactivation. A computed tomography (CT) scan to evaluate the leukemic tumor showed bilateral interstitial pneumonitis on day 33, although he had no respiratory symptoms. The findings of the CT scan were consistent with those of HHV-6 pneumonitis that were reported previously. HHV-6 DNA, but no other pathogens, was detected in his bronchoalveolar lavage (BAL) fluid. The patient was successfully treated with a therapeutic dose of foscarnet. This case indicates that performing a CT scan around the time of neutrophil engraftment can play an important role in detecting the early phase of HHV-6 pneumonia, and BAL should be considered if features consistent with HHV-6 pneumonitis are observed in patients with a risk of HHV-6 reactivation. Takashi Ishio, Tomoyuki Endo, Kohei Okada, Akio Shigematsu, Satoshi Hashino, and Takanori Teshima Copyright © 2015 Takashi Ishio et al. All rights reserved. Sirolimus-Based Immunosuppression as GvHD Prophylaxis after Bone Marrow Transplantation for Severe Aplastic Anaemia: A Case Report and Review of the Literature Mon, 05 Jan 2015 13:58:49 +0000 Congenital or acquired severe aplastic anaemia (SAA) is cured by bone marrow transplantation (BMT) from a histocompatible leukocyte antigen- (HLA-) identical sibling. The best conditioning regimen is cyclophosphamide (CTX) with or without antithymocyte globulin (ATG), followed by short-term methotrexate (MTX) and cyclosporine A (CsA) to prevent graft-versus-host disease (GvHD). In our pediatric oncology-hematology unit, a 5-year-old girl with SAA was treated with two BMT from the same HLA-identical sibling donor. Severe CsA-induced adverse events (severe hypertension and PRES) after the first BMT led necessarily to CSA withdrawal. Alternative immunosuppressive treatment for GvHD prevention as tacrolimus and mycophenolate were not tolerated by our patient because toxicity > grade II. For this reason we decided to administrate sirolimus alone as GvHD prophylaxis and to prevent disease relapse after the rescue BMT. Here we report the successful use of sirolimus alone for GvHD prophylaxis after the second transplant in a pediatric BMT setting for SAA. Katia Perruccio, Elena Mastrodicasa, Francesco Arcioni, Ilaria Capolsini, Carla Cerri, Grazia Gurdo, and Maurizio Caniglia Copyright © 2015 Katia Perruccio et al. All rights reserved. Primary Hepatic Lymphoma in a Patient with Rheumatoid Arthritis Treated with Methotrexate Wed, 24 Dec 2014 09:24:10 +0000 Primary hepatic lymphoma (PHL) has rarely been reported in patients with immunosuppression. We herein describe a case of Epstein-Barr virus- (EBV-) positive PHL in a 67-year-old Japanese woman receiving methotrexate (MTX) treatment for rheumatoid arthritis (RA). The patient, who had been receiving MTX therapy for more than 6 years, presented with low-grade fever and abdominal pain. Initial laboratory tests showed mildly elevated liver enzymes with normal levels of alpha-fetoprotein and carcinoembryonic antigen, and computed tomography scans revealed multiple hepatic tumors with no lymph-node swelling. Examination of liver specimens obtained via ultrasonography-guided needle biopsy indicated EBV-positive diffuse large B cell lymphoma; therefore, she was diagnosed with PHL. MTX was discontinued, and she was carefully monitored thereafter owing to the prolonged history of MTX administration for RA. Rapid progression of PHL was observed; therefore 10 days after the PHL diagnosis, she received 6 cycles of R-THP-COP (rituximab, cyclophosphamide, pirarubicin, vincristine, and prednisolone) therapy and achieved complete remission for more than 1 year. Although MTX-associated lymphoproliferative disorders often show remission after withdrawal of MTX, early diagnosis and treatment are essential for PHL in patients with RA treated with MTX, because of the aggressive nature of the disease. Goichi Tatsumi, Naoya Ukyo, Hirokazu Hirata, and Mitsuru Tsudo Copyright © 2014 Goichi Tatsumi et al. All rights reserved. Adult T-Cell Lymphoma/Leukemia Presenting as Isolated Central Nervous System T-Cell Lymphoma Tue, 23 Dec 2014 10:39:18 +0000 Adult T-cell leukemia/lymphoma (ATLL) is a T-cell neoplasm, associated with infection by the retrovirus human T-lymphotropic virus type 1 (HTLV-1). Central nervous system (CNS) involved by ATLL is often occurred in advanced disease, such as acute and lymphomatous variants. On the other hand, isolated CNS lymphoma is rare. We repot a 50-year-old woman who presented with multiple infiltrative brain lesions on the magnetic resonance (MR) imaging. Results of initial biopsy of brain tumor indicated CNS vasculitis. The patient received one course of high-dose methotrexate and MR imaging of brain revealed remission of infiltrative lesions. Two years later, new brain lesions were detected. Histopathologic examination of specimens via craniotomy revealed T-cell lymphoma. The patient responded poorly to subsequent chemotherapy, and salvage whole-brain irradiation was performed. Six months later, the patient had hepatosplenomegaly, hypercalcemia, and multiple lymphocytes with a cloverleaf appearance in circulation. Results of flow cytometry analysis of peripheral blood indicated ATLL and antibodies to human T-lymphotropic virus type 1 (HTLV-1) were detected. Clinicians should screen HTLV-1 infection when patients are diagnosed with peripheral T-cell lymphoma. Combined antiviral therapy and intensive chemotherapy may improve the outcomes of ATLL. Wei-Li Ma, Chi-Cheng Li, Shan-Chi Yu, and Hwei-Fang Tien Copyright © 2014 Wei-Li Ma et al. All rights reserved. Polyangiitis with Granulomatosis as a Paraneoplastic Syndrome of B-Cell Lymphoma of the Lacrimal Gland Thu, 18 Dec 2014 00:10:47 +0000 Introduction. The clinical course of an autoimmune paraneoplastic syndrome parallels the natural history of the primary malignancy. In most cases, such paraneoplastic are syndromes hardly distinguishable from idiopathic autoimmune diseases. A case of polyangiitis with granulomatosis as a paraneoplastic syndrome in a patient with B-cell Lymphoma of the lacrimal gland has not yet been reported. Case Presentation. We present the case of a male patient with a B-cell Lymphoma of the lacrimal gland, who debuted with symptoms similar to rheumatoid arthritis and acute renal failure, secondary to polyangiitis with granulomatosis. The current pathophysiological hypotheses explaining the relationship between a lymphoproliferative disease and an autoimmune paraneoplastic disorder are discussed. Conclusion. Tumor-associated segmental necrotizing glomerulopathy is a very rare manifestation of glomerular diseases. Some atypical clinical features should increase the suspicion of an underlying tumor, in which case it is essential to treat the primary neoplasia, in order to control the autoimmune manifestations. Beatriz Wills Sanín, Yenny R. Cárdenas Bolivar, Jose J. Carvajal, Guillermo E. Quintero, and Rafael Andrade Copyright © 2014 Beatriz Wills Sanín et al. All rights reserved. Skin Recurrence of Transformed Mycosis Fungoides Postumbilical Cord Blood Transplant despite Complete Donor Chimerism Thu, 11 Dec 2014 12:49:23 +0000 Background. Allogeneic stem cell transplant is the treatment of choice for systemic cutaneous T-cell lymphoma (CTCL) which provides graft-versus-lymphoma effect. Herein we discuss a case of recurrence of CTCL skin lesions after cord blood transplant in a patient who continued to have 100% donor chimerism in bone marrow. Case Presentation. A 48-year-old female with history of mycosis fungoides (MF) presented with biopsy proven large cell transformation of MF. PET scan revealed multiple adenopathy in abdomen and chest suspicious for lymphoma and skin biopsy showed large cell transformation. She was treated with multiple cycles of chemotherapy. Posttherapy PET scan showed resolution of lymphadenopathy. Later she underwent ablative preparative regimen followed by single cord blood transplant. Bone marrow chimerism studies at day +60 after transplant showed 100% donor cells without presence of lymphoma. However 5 months after transplant she had recurrence of MF with the same genotype as prior skin lesion. Bone marrow chimerism study continued to show 100% donor cells. Conclusion. A differential graft-versus-lymphoma effect in our case prevented lymphoma recurrence systemically but failed to do so in skin. We hypothesize that this response may be due to presence of other factors in the bone marrow and lymph node microenvironments preventing recurrence in these sites. Rahul Pawar, Anup Kasi Loknath Kumar, Janet Woodroof, Wei Cui, Joseph McGuirk, Sunil Abhyankar, Sid Ganguly, Anurag Singh, Tara Lin, and Omar Aljitawi Copyright © 2014 Rahul Pawar et al. All rights reserved. Visceral Leishmaniasis: A Differential Diagnosis to Remember after Bone Marrow Transplantation Thu, 11 Dec 2014 07:47:23 +0000 Leishmania infection in immunocompromised hosts is reported in the literature, mostly concerning human immunodeficiency virus infected patients. It is not well characterized in the context of stem cell transplantation. We report a rare case clinical case of visceral leishmaniasis after allogeneic bone marrow transplantation. A 50-year-old Caucasian male was referred to allogeneic bone marrow transplantation with a high-risk acute lymphoblastic B leukemia in first complete remission. Allogeneic SCT was performed with peripheral blood stem cells from an unrelated Portuguese matched donor. In the following months, patient developed mild fluctuating cytopenias, mostly thrombocytopenia (between 60 and 80∗109/L). The only significant complaint was intermittent tiredness. The common causes for thrombocytopenia in this setting were excluded—no evidence of graft versus host disease, no signs of viral or bacterial infection, and no signs of relapsed disease/dysplastic changes. The bone marrow smear performed 12 months after transplantation revealed an unsuspected diagnosis: a massive bone marrow infiltration with amastigotes. Margarida Dantas Brito, Fernando Campilho, Rosa Branca, Carlos Pinho Vaz, Cristina Silva, Teresa Sousa, Carlos Mendes, and António Campos Copyright © 2014 Margarida Dantas Brito et al. All rights reserved. Acquired Von Willebrand’s Syndrome in Systemic Lupus Erythematosus Sun, 07 Dec 2014 00:10:32 +0000 Acquired von Willebrand syndrome (AVWS) is an uncommon, underdiagnosed, and heterogeneous disease which is increasingly recognized as a cause of bleeding diatheses. Systemic lupus erythematosus (SLE) is an infrequent cause of AVWS. Herein, we report a case of AVWS diagnosed during the initial presentation of SLE in a previously healthy young man with no family history of bleeding diathesis who presented with worsening epistaxis, gastrointestinal bleeding, and anasarca. He was found to have severe anemia and prolonged activated partial thromboplastin time (aPTT) with severely decreased levels of von Willebrand factor (VWF) measurements in addition to markedly decreased factor VIII levels. Further evaluation revealed nephrotic syndrome and interstitial lung disease due to SLE. He initially received combination therapy with intravenous immunoglobulin (IVIG) and von Willebrand factor/factor VIII concentrates without significant improvement. Treatment with steroids, cyclophosphamide, and rituximab was followed by clinical improvement evidenced by cessation of bleeding. The short follow-up did not allow us to definitely prove the therapeutic effect of immunosuppressive treatment on AVWS in SLE patients. This case adds to the literature supporting the relationship between AVWS and SLE and highlights the importance of combination therapy in the treatment of severe AVWS as well as the role of IVIG, cyclophosphamide, and rituximab in AVWS associated with SLE. Sara Taveras Alam, Karenza Alexis, Ashwin Sridharan, Marianna Strakhan, Tarek Elrafei, Richard J. Gralla, and Louis J. Reed Copyright © 2014 Sara Taveras Alam et al. All rights reserved. Follicular Lymphoma Presenting with Leptomeningeal Disease Tue, 02 Dec 2014 11:40:05 +0000 Follicular lymphoma is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Central nervous system metastasis is a very rare complication portending a very poor prognosis. We report a rare case of follicular lymphoma presenting with leptomeningeal involvement achieving a complete remission after initial therapy. Rubens Costa, Ricardo Costa, and Renata Costa Copyright © 2014 Rubens Costa et al. All rights reserved. Oral Lesion as Unusual First Manifestation of Multiple Myeloma: Case Reports and Review of the Literature Tue, 25 Nov 2014 00:00:00 +0000 Extramedullary plasmacytoma (EMP) and solitary bone plasmacytoma (SBP) represent a disease continuum through a multistage process of cell differentiation, survival, proliferation, and dissemination, strictly related to multiple myeloma (MM), the second most common hematological malignancy. Herein, we report two cases of recurrent oral plasmacytoma progressed to MM, in which the first clinical sign of a more widespread disease was limited to the mouth. Based on our experience, we recommend a strict workup for the differential diagnosis between EMP, SBP, and MM for patients with oral plasmacytoma, including radiological exam of the skeleton, magnetic resonance imaging (MRI) of the bone, and positive emission tomography (FDG-PET). MRI and possibly PET can all be used to more sensitively detect EM plasmacytoma sites. A. Romano, M. S. Marescalco, Chiara Liardo, L. Villari, C. Vetro, C. Conticello, F. Di Raimondo, and S. Ferlito Copyright © 2014 A. Romano et al. All rights reserved. Clinical Lessons to Be Learned from Patients Developing Chronic Myeloid Leukemia While on Immunosuppressive Therapy after Solid Organ Transplantation: Yet Another Case after Orthotopic Heart Transplantation Sun, 16 Nov 2014 13:32:20 +0000 Chronic myeloid leukemia developing after transplantation of solid organs and concomitant immunosuppression is a rare but still significant clinical phenomenon. We here describe an additional case of a 62-year-old male patient developing CML after orthotopic heart transplantation and medication with cyclosporine A, mofetil-mycophenolate, and steroids. Initial antileukemic therapy was imatinib at a standard dose and within 15 months of therapy a complete cytogenetic response was noted. In this report we discuss the clinical implications of these rare but biologically important cases. Christian Oberender, Lorenz Kleeberg, Nicola Nienhues, Martin Gresse, Bernd Dörken, Hanno Riess, and Philipp le Coutre Copyright © 2014 Christian Oberender et al. All rights reserved. A Rare Presentation of In Situ Mantle Cell Lymphoma and Follicular Lymphoma: A Case Report and Review of the Literature Sun, 16 Nov 2014 08:57:46 +0000 A 65-year-old gentleman presented with left groin swelling over the course of two months. Physical exam revealed nontender left inguinal adenopathy, and computed tomography scans detected multiple lymph nodes in the mesenteric, aortocaval, and right common iliac regions. An excisional lymph node biopsy was performed. Pathologic evaluation demonstrated follicular center site which stained positive for PAX5, CD20, CD10, Bcl-2, Bcl-6, and mantle zone cells. These findings demonstrated CCND1 and CD5 positivity, suggesting composite lymphoma comprising follicular lymphoma (FL) with in situ mantle cell lymphoma (MCLIS). FL is known as indolent non-Hodgkin lymphoma; however, the clinical significance of a coexisting MCLIS continues to be elusive, and optimal management of these patients remains largely unknown. This case illustrates the diagnostic and therapeutic challenges of composite lymphomas. This paper also discusses advances in molecular pathogenesis and lymphoma genomics which offer novel insights into these rare diseases. Josephine Taverna, Anju Nair, Seongseok Yun, Spencer Paulson, Jonathan H. Schatz, Daniel Persky, Deborah Fuchs, and Soham Puvvada Copyright © 2014 Josephine Taverna et al. All rights reserved. Managing a Rivaroxaban Bleed: Understanding the Difficulties in Acute Reversal of the New Oral Anticoagulants through a Case Report Thu, 13 Nov 2014 09:41:17 +0000 With the arrival of a new generation of oral anticoagulants significant burdens associated with warfarin’s use on both the patient and the healthcare system have been alleviated. Nevertheless, a shortfall exists in regard to an agent or protocol for reversal of these new anticoagulants in the setting of an acute bleed. Our case of a patient presenting to the hospital with a vaginal bleed while on rivaroxaban highlights the difficulty in management without a clear protocol or agent for reversal of anticoagulation. Naveen Nannapaneni, Robby Singh, Paulina Mckay, and Marwan Al-Hajeili Copyright © 2014 Naveen Nannapaneni et al. All rights reserved. Familial Essential Thrombocythemia Associated with MPL W515L Mutation in Father and JAK2 V617F Mutation in Daughter Mon, 10 Nov 2014 12:03:49 +0000 Familial essential thrombocythemia features the acquisition of somatic mutations and an evolution similar to the sporadic form of the disease. Here we report two patients—father and daughter—with essential thrombocythemia who displayed a heterogeneous pattern of somatic mutations. The JAK2 V617F mutation was found in the daughter, while the father harbored the MPL W515L mutation. This case report may constitute further proof that in familial essential thrombocythemia there are other, still undefined, constitutional, inherited genetic factors predisposing to the acquisition of various somatic mutations (e.g., JAK2 V617F and MPL). Adrian P. Trifa, Andrei Cucuianu, and Radu A. Popp Copyright © 2014 Adrian P. Trifa et al. All rights reserved. Chronic Idiopathic Neutrophilia in Two Twins Thu, 06 Nov 2014 13:09:32 +0000 Neutrophilia in adults refers to an alteration in the total number of blood neutrophils that is in excess of about 7500 cells/μL. This definition is restrictive in childhood as neutrophil count is age-dependent. Chronic Idiopathic Neutrophilia (CIN) refers to a condition that persists for many years in individuals who appear otherwise healthy. CIN is rarely mentioned in scientific literature and in academic books of hematology; only few words are dedicated to this topic. We report a case study of two twins with CIN followed from the first year of life to 24 years of age. To the best of our knowledge this is the first case report of two twins with CIN followed through a long period of time. We believe that our observation may contribute to better understand and characterize this hematologic abnormality. Roberto Miniero, Giuseppe Antonio Mazza, Federica Altomare, and Carla Fusaro Copyright © 2014 Roberto Miniero et al. All rights reserved. Primary Cardiac Burkitt Lymphoma Presenting with Abdominal Pain Thu, 06 Nov 2014 13:04:54 +0000 We describe the case of a 44-year-old woman with primary Burkitt lymphoma of the heart who presented with abdominal bloating and epigastric discomfort secondary to tamponade physiology caused by a large pericardial effusion. The pericardial fluid contained a large number of highly atypical lymphocytes with moderate basophilic cytoplasm, rare punched-out vacuoles, a vesicular nuclear chromatin, large nucleolus, and marginated chromatin that by FISH were positive for the 8;14 translocation. She had no other sites of disease. She was treated with four alternating cycles of modified CODOX-M and IVAC in combination with rituximab and remains in remission more than 5 years since diagnosis. Dimitrios Tzachanis, Rajan Dewar, Katarina Luptakova, James D. Chang, and Robin M. Joyce Copyright © 2014 Dimitrios Tzachanis et al. All rights reserved. Acquired Factor XI Inhibitor Presenting as Spontaneous Bilateral Subdural Hematoma in an Elderly Patient Thu, 06 Nov 2014 07:16:28 +0000 Development of autoantibodies against coagulation factors is an uncommon bleeding disorder associated with cancer, autoimmune conditions, pregnancy, or no apparent disease. Spontaneous FVIII inhibitors are the most frequently encountered; those against FXI have been only anecdotally reported. We report a case of acquired FXI inhibitor presenting as fatal intracranial spontaneous bleeding in an elderly patient with history of cancer and previous transfusions. Few cases of acquired FXI inhibitor have been reported in association with connective tissue disease, cancer, or surgery. Bleeding includes mucocutaneous bleeding, postsurgical hemorrhage, or life-threatening events. Treatment consists of arresting the bleeding and inhibitor eradication. High degree of suspicion is essential to promptly diagnose and treat this uncommon condition. Natale Vazzana, Luca Scarti, Chiara Beltrame, Antonella Picchi, Gianni Taccetti, and Alberto Fortini Copyright © 2014 Natale Vazzana et al. All rights reserved. Hemophagocytic Lymphohistiocytosis in Association with Primary Cutaneous Anaplastic Large Cell Lymphoma Tue, 28 Oct 2014 06:23:46 +0000 Hemophagocytic lymphohistiocytosis (HLH) has a well known association with lymphomas, especially of T cell origin. Prognosis of lymphoma associated HLH is very poor, especially in T cell lymphomas; and, therefore, early diagnosis might alter the outcome. Though association of HLH with systemic anaplastic large cell lymphoma (ALCL) is known, its occurrence in primary cutaneous ALCL (C-ALCL) is distinctly rare. We aim to describe a case of C-ALCL (anaplastic lymphoma kinase (ALK)−) in an elderly male who succumbed to the complication of associated HLH, which was possibly triggered by coexistent virus infection. We briefly present the literatures on lymphoma associated HLH and discuss the histopathological differentials of cutaneous CD30+ lymphoproliferative disorders. We do suggest that HLH may pose diagnostic challenges in the evaluation of an underlying lymphoma and hence warrants proper evaluation for the underlying etiologies and/or triggering factors. Aneesh Basheer, Somanath Padhi, Ramesh Nagarajan, Vinoth Boopathy, Sudhagar Mookkappan, and Nayyar Iqbal Copyright © 2014 Aneesh Basheer et al. All rights reserved. Eltrombopag in Good’s Syndrome Sun, 19 Oct 2014 10:34:30 +0000 Good’s syndrome is a rare acquired immunodeficiency associated with thymoma. Eltrombopag is a thrombopoietin receptor agonist and has been shown to be a valuable supplement to the treatment of several types of refractory cytopenias. In this paper, we describe a male patient suffering from Good’s syndrome with immune-mediated T-cell driven pancytopenia and absence of megakaryopoiesis. He was successfully treated with eltrombopag resulting in a multilineage clinical response. Håvard Anton Kristiansen, Signe Spetalen, Yngvar Fløisand, and Dag Heldal Copyright © 2014 Håvard Anton Kristiansen et al. All rights reserved. A Unique Case of Relapsed B-Acute Lymphoblastic Leukemia/Lymphoma as an Isolated Omental Mass Thu, 16 Oct 2014 11:44:01 +0000 B-acute lymphoblastic leukemia/lymphoma (B-ALL) is a neoplasm of precursor cells committed to the B-cell lineage. Extramedullary involvement is frequent, with particular predilection for the central nervous system, lymph nodes, spleen, liver, and testis. We report an unusual case of B-ALL relapsing as an isolated omental mass along with bone marrow involvement. Kanchan Kantekure, Furha Cossor, Kenneth B. Miller, and Monika E. Pilichowska Copyright © 2014 Kanchan Kantekure et al. All rights reserved. 36-Year-Old Female with Catastrophic Antiphospholipid Syndrome Treated with Eculizumab: A Case Report and Review of Literature Wed, 15 Oct 2014 11:26:39 +0000 Catastrophic antiphospholipid syndrome (CAPS) is a rare but potentially life-threatening condition characterized by diffuse vascular thrombosis, leading to multiple organ failure developing over a short period of time in the presence of positive antiphospholipid antibodies (aPL). CAPS is a severe form of antiphospholipid syndrome, developing in about 1% of cases of classic antiphospholipid syndrome, manifesting as microangiopathy, affecting small vessels of multiple organs. It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation. Lupus anticoagulant and anticardiolipin antibodies have been reported as predominant antibodies associated with CAPS. Treatment options often utilized in CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%. The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition. Studies have shown that complement activation plays a key role in the pathogenesis of aPL mediated thrombosis in CAPS. We report a case of a 36-year-old female admitted with clinical and laboratory findings consistent with CAPS successfully treated with eculizumab, a terminal complement inhibitor. Marianna Strakhan, Mariana Hurtado-Sbordoni, Nahun Galeas, Kamila Bakirhan, Karenza Alexis, and Tarek Elrafei Copyright © 2014 Marianna Strakhan et al. All rights reserved. Unusual Case of Simultaneous Presentation of Plasma Cell Myeloma, Chronic Myelogenous Leukemia, and a Jak2 Positive Myeloproliferative Disorder Wed, 15 Oct 2014 09:42:23 +0000 Background. Multiple articles discuss the rare incidence and potential causes of second hematologic disorders arising after treatment of Chronic Myelogenous Leukemia (CML), leading to the theory of imatinib, the current treatment regimen for CML, as a possible trigger for the development of secondary neoplasms. Our case eliminates the possibility of imatinib as the sole cause since our patient received a diagnosis of simultaneous plasma cell myeloma, CML, and a Jak2 mutation positive myeloproliferative disorder (MPD) arising de novo, prior to any treatment. We will further investigate into alternative theories as potential causes for multiple hematopathologic disorders. Case Report. There are currently no reported cases with the diagnosis of simultaneous plasma cell myeloma, chronic myelogenous leukemia, and Jak2 positive myeloproliferative disorder. We present a case of a 77-year-old male who was discovered to have these three concurring hematopathologic diagnoses. Our review of the literature includes a look at potential associations linking the three coexisting hematologic entities. Conclusion. The mechanism resulting in simultaneous malignancies is most likely multifactorial and potentially includes factors specific to the host, continuous stimulation of the immune system, previous chemotherapy or radiation, a potential common pluripotent stem cell, or, lastly, preexisting myeloma which may increase the susceptibility of additional malignancies. J. Maerki, G. Katava, D. Siegel, J. Silberberg, and P. K. Bhattacharyya Copyright © 2014 J. Maerki et al. All rights reserved. A Jehovah’s Witness with Acute Myeloid Leukemia Successfully Treated with an Epigenetic Drug, Azacitidine: A Clue for Development of Anti-AML Therapy Requiring Minimum Blood Transfusions Thu, 02 Oct 2014 09:28:45 +0000 Therapy for acute leukemia in Jehovah’s Witnesses patients is very challenging because of their refusal to accept blood transfusions, a fundamental supportive therapy for this disease. These patients are often denied treatment for fear of treatment-related death. We present the first Jehovah’s Witness patient with acute myeloid leukemia (AML) treated successfully with azacitidine. After achieving complete remission (CR) with one course of azacitidine therapy, the patient received conventional postremission chemotherapy and remained in CR. In the case of patients who accept blood transfusions, there are reports indicating the treatment of AML patients with azacitidine. In these reports, azacitidine therapy was less toxic, including hematoxicity, compared with conventional chemotherapy. The CR rate in azacitidine-treated patients was inadequate; however, some characteristics could be useful in predicting azacitidine responders. The present case is useful for treating Jehovah’s Witnesses patients with AML and provides a clue for anti-AML therapy requiring minimum blood transfusions. Yumi Yamamoto, Akihito Kawashima, Eri Kashiwagi, and Kiyoyuki Ogata Copyright © 2014 Yumi Yamamoto et al. All rights reserved. A Double Hit CD10-Negative B-Cell Lymphoma with t(3;8)(q27;q24) Leading to Juxtaposition of the BCL6 and MYC Loci Associated with Good Clinical Outcome Wed, 01 Oct 2014 11:30:39 +0000 The WHO classification of lymphomas allows for a group of diseases that have features intermediate between those of Burkitt lymphoma and diffuse large B-cell lymphoma. These are a diverse group of diseases whose genetics and clinical course are yet to be fully described. We report an unusual case of high grade B-cell lymphoma, intermediate between DLBCL and BL, lacking CD10 expression in which the chromosomal translocation t(3;8)(q27;q24) was found to be the sole chromosomal abnormality. FISH analysis demonstrated juxtaposition of the BCL6 and MYC loci without obvious involvement of the IGH locus, suggesting constitutive MYC expression due to promoter substitution. The patient responded to intensive chemotherapy and remains in remission two years after finishing therapy. Lucinda Sanders, Sandrine Jayne, Ben Kennedy, Fiona Miall, Sietse M. Aukema, Reiner Siebert, Simon D. Wagner, and Martin J. S. Dyer Copyright © 2014 Lucinda Sanders et al. All rights reserved. Autoimmune Demyelinating Polyneuropathy as a Manifestation of Chronic Graft-versus-Host Disease after Adult Cord Blood Transplantation in a Patient with Chronic Lymphocytic Leukemia Sun, 14 Sep 2014 09:17:24 +0000 Immune mediated demyelinating disease after allogeneic stem cell transplantation is a rare entity with unclear etiology. Acute inflammatory demyelinating polyneuropathy (AIDP) has been reported after related and adult unrelated allogeneic stem cell transplantation but no such case has been reported after unrelated cord blood transplantation. We hereby present the first case of AIDP after double umbilical cord blood transplantation (DUCBT). A 55-year-old man with chronic lymphocytic leukemia (CLL) received a cord blood transplant for relapsed refractory disease with high risk cytogenetics. On day 221, patient presented with skin rash, tingling in both lower extremites, and ascending paralysis that progressed rapidly over the course of 2 days. The workup resulted in a diagnosis of AIDP and administration of intravenous immunoglobulins plus steroids was initiated. Motor and sensory powers were fully recovered and his chronic GVHD was managed for several months with single agent sirolimus. Fredrick Hogan and Melhem Solh Copyright © 2014 Fredrick Hogan and Melhem Solh. All rights reserved. Myeloid Sarcoma Presenting with Leukemoid Reaction in a Child Treated for Acute Lymphoblastic Leukemia Wed, 03 Sep 2014 08:38:02 +0000 Background. Myeloid sarcoma is an extramedullary neoplasm of immature myeloid cells. Our study reports a presentation of myeloid sarcoma which presented with severe leukemoid reaction as a secondary malignancy in a patient who was treated for acute lymphoblastic leukemia previously. The case emphasizes the difficulties in diagnosis of patients who do not have concomitant leukemia. Case Presentation. A 6-year-old girl who was treated for acute lymphoblastic leukemia previously presented with fatigue, paleness, and hepatosplenomegaly. Peripheral blood smear and bone marrow aspirate examination did not demonstrate any blasts in spite of severe leukemoid reaction with a white cell count 158000/mm3. FDG/PET CT revealed slight uptake in cervical and supraclavicular lymph nodes. Excisional lymph node biopsy was performed from these lymph nodes and it showed myeloid sarcoma. Conclusion. Myeloid sarcoma can develop as a secondary malignancy in children who are treated for acute lymphoblastic leukemia. It can be associated with severe leukemoid reaction and diagnosis may be difficult if there is not concomitant leukemia. PET/CT is helpful in such cases. Aylin Canbolat Ayhan, Cetin Timur, Seyma Sonmez, Ebru Zemheri, and Asım Yoruk Copyright © 2014 Aylin Canbolat Ayhan et al. All rights reserved. Chromothripsis: Basis of a Concurrent Unusual Association between Myelodysplastic Syndrome and Primary Ciliary Dyskinesia Mon, 01 Sep 2014 05:39:28 +0000 A 20 year old male was initially diagnosed suffering from Primary ciliary dyskinesia with symptoms of bronchiectasis, severe frontal, maxillary and ethmoid sinus disease. At the age of 20, the patient was also diagnosed with Myelodysplastic syndrome requiring Bone marrow transplant due to the advanced stage at time of presentation. Primary ciliary dyskinesia and Myelodsyplastic syndrome are both rare clinical conditions found in the general population, especially in young adults. This rare combination of disorders has never been reported in literature to the best of the author’s knowledge. The presence of an advanced cancer and a genetic abnormality due to two deletions occurring in two arms of the same chromosome can be explained on the base of chromothripsis. A number of evidences have been published in the literature, about multiple deletions in chromosome 5 and advanced stages of MDS being associated with chromothripsis however this is the first case report on two deletions in chromosome 7 giving rise to two different clinical entities requiring multiple modes of management. Abhinav Agrawal, Anar Modi, Sayee Sundar Alagusundaramoorthy, and Wael Ghali Copyright © 2014 Abhinav Agrawal et al. All rights reserved. CNS Involvement in AML Patient Treated with 5-Azacytidine Thu, 14 Aug 2014 12:15:29 +0000 Central nervous system (CNS) involvement in acute myeloid leukemia (AML) is a rare complication of the disease and is associated with poor prognosis. Sometimes the clinical presentation can be unspecific and the diagnosis can be very challenging. Here we report a case of CNS infiltration in a patient suffering from AML who presented with normal complete blood count and altered mental status. Diamantina Vasilatou, Sotirios Papageorgiou, Efthymia Bazani, Athina Prasouli, Christina Economopoulou, Christoforos Roumpakis, Petros Karakitsos, George Dimitriadis, and Vasiliki Pappa Copyright © 2014 Diamantina Vasilatou et al. All rights reserved. Two Cases of Q-Fever in Hairy Cell Leukemia Tue, 12 Aug 2014 00:00:00 +0000 Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder accounting for about 2% of all leukemias. The clinical course is indolent, however HCL patients are particularly susceptible to infections. Here we report two cases of Q-fever as first manifestation of disease in two patients affected by HCL. Both patients described in this report showed an unusually sluggish clinical response to the antibiotic treatment with ciprofloxacin probably because of the marked immunodeficiency. However, treatment of HCL with cladribine administered soon after the resolution of QF pneumonitis was uneventful and led to a complete remission in both cases. Most probably the association of Coxiella burnetii (CB) infection and HCL that we observed in two patients is due to chance. However, a hairy cell resembling transformation of freshly isolated human peripheral blood lymphocytes upon CB has been showed. We think that the possibility of CB infection in febrile HCL patient should be always taken in mind, especially in endemic areas. In addition the potential for such infections to become chronic in HCL patients should not be overlooked and the reporting of further cases should be encouraged. Emanuele Ammatuna, Emilio Iannitto, Lidwine W. Tick, Nicolaas L. A. Arents, Philip H. Kuijper, and Marten R. Nijziel Copyright © 2014 Emanuele Ammatuna et al. All rights reserved.