Case Reports in Hematology https://www.hindawi.com The latest articles from Hindawi © 2017 , Hindawi Limited . All rights reserved. An Unsuspected Finding of t(9;22): A Rare Case of Philadelphia Chromosome-Positive B-Lymphoblastic Lymphoma Mon, 18 Sep 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/2413587/ While rare, cases of isolated extramedullary disease of B-cell Lymphoblastic Lymphoma (B-LBL) without morphologic bone marrow involvement have been described. In this report, we illustrate the case of an elderly gentleman who presented with isolated testicular and vertebral LBL involvement but had no morphologic bone marrow involvement. The initial plan of treatment was to treat along the lines of Philadelphia negative B-ALL/LBL. During this time, fluorescence in situ hybridization (FISH) and PCR testing for BCR-ABL1 rearrangements were being performed on the marrow specimens as a part of routine diagnostic workup. While the FISH returned negative, PCR testing unexpectedly detected BCR-ABL1 fusion transcripts at a low level of 0.48%. Given their presence, we performed FISH for BCR/ABL1 rearrangement in both testicular and L5 vertebral specimens which were 80–90% positive. He subsequently received rituximab, hyper-CVAD, and dasatinib, along with prophylactic intrathecal prophylactic chemotherapy. The patient achieved a prolonged remission but eventually relapsed, 4 years later. Had it not been for this fortuitous discovery, the patient would not have been treated with tyrosine kinase inhibitors. We emphasize that FISH and PCR testing for BCR-ABL1 rearrangement are integral to arriving at an accurate diagnosis and should be routinely tested on B-LBL biopsy specimens. Prajwal Boddu, C. Cameron Yin, Rashmi Kanagal-Shamanna, Guillin Tang, Beenu Thakral, Tapan Kadia, Marina Konopleva, Elias Jabbour, and Nitin Jain Copyright © 2017 Prajwal Boddu et al. All rights reserved. HbS-Sicilian (δβ)0-Thalassemia: A Rare Variant of Sickle Cell Sun, 17 Sep 2017 08:41:13 +0000 http://www.hindawi.com/journals/crihem/2017/9265396/ Sickle cell disease (SCD) is caused by a mutation in the sixth codon of the -globin gene on chromosome 11, which leads to a single amino acid substitution (glutamine to valine). Sickle-(δβ)0-thalassemia is a rare variant of sickle cell disease (delta-beta thalassemia occurring in association with sickle hemoglobin, HbS), sparsely reported in literature, and has been associated with symptomatology necessitating careful monitoring and follow-up. We describe a patient who presented with a newborn screen reported as “FS” and a negative family history for sickle cell disease and sickle cell trait. Subsequent gene sequencing studies demonstrated the presence of Sickle-(δβ)0-thalassemia. Clinical course has remained relatively stable for this patient now at 18 months of age without any SCD related symptomatology or complications. As this is a rare variant of SCD with potential complications, it is important to establish diagnosis towards planning comprehensive care. Grace Onimoe and Genine Smarzo Copyright © 2017 Grace Onimoe and Genine Smarzo. All rights reserved. Cutaneous Vasculitis: An Unusual Presentation of a Biclonal Nodal Plasma Cell Dyscrasia Wed, 13 Sep 2017 10:05:22 +0000 http://www.hindawi.com/journals/crihem/2017/8152610/ We describe an unusual case of a biclonal nodal plasma cell dyscrasia, presenting with a vasculitic rash, end-organ damage, and cytopenias. Serum protein electrophoresis demonstrated a biclonal kappa-restricted paraprotein, with a negative skeletal survey and no bone marrow disease. Fluorodeoxyglucose-PET-CT (FDG-PET-CT) revealed nodal involvement, which was not appreciable clinically, and facilitated biopsy, confirming the diagnosis of a nodal plasmacytoma. Complete biochemical response and resolution of the vasculitic rash were achieved with bortezomib-based therapy. D. Swan, M. Murphy, and E. Elhassadi Copyright © 2017 D. Swan et al. All rights reserved. Posttransplant Lymphoproliferative Disorder in a Patient with Worsening Ascites after Liver Transplantation Mon, 11 Sep 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/7247438/ Posttransplant lymphoproliferative disorder (PTLD) is a spectrum of diseases that involves abnormal lymphoid and/or plasmacytic proliferation in patients with solid organ or hematopoietic cell transplantation. It is a condition with a low incidence of 3.5–4.3% in liver transplant (LT) recipients. This case involves a 63-year-old male with history of LT for chronic HCV induced cirrhosis who presented with abdominal distension related to worsening ascites. Cytological ascitic fluid analysis revealed EBV (+) malignant cells without a malignant focal point on imaging. Diagnosis of monomorphic PTLD with primary effusion lymphoma-like morphology and immunophenotype was established. This case highlights the complexity in diagnosis, different diagnostic modalities, and rare clinical presentations of PTLD. Harsh D. Patel and Moises I. Nevah Rubin Copyright © 2017 Harsh D. Patel and Moises I. Nevah Rubin. All rights reserved. Immune-Mediated Autonomic Neuropathies following Allogeneic Stem Cell Transplantation in Acute Myeloid Leukemia Sun, 27 Aug 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/6803804/ Background/Aims. Autonomic dysfunction (AD) after allogeneic stem cell transplant (SCT) is a rare occurrence and likely immune-mediated in etiology. There is limited literature on this topic and hence, we wish to briefly describe management of two cases at our institution and their outcomes. Methods. We retrospectively identified two patients with immune-mediated AD after SCT from our database. Immune-mediated AD was defined as AD secondary to an immune-mediated etiology without an alternative cause and responding to immunosuppression. Results. The first case is of a 32-year-old man with acute myeloid leukemia (AML) who underwent double umbilical cord allogeneic SCT. The second patient was a 51-year-old woman with secondary AML who underwent matched-related donor allogeneic SCT. Both underwent an extensive work-up for an underlying etiology prior to treatment with intravenous immunoglobulin (IVIG). Conclusions. AD after SCT is a rare yet significant clinical entity. A work-up of underlying etiology should be performed. IVIG is a treatment option for these patients. Abhishek Mangaonkar, Hassan Al Khateeb, Narjust Duma, Erik K. St. Louis, Andrew McKeon, Mrinal Patnaik, William Hogan, Mark Litzow, and Taxiarchis Kourelis Copyright © 2017 Abhishek Mangaonkar et al. All rights reserved. Acute Unilateral Renal Infarction in the Setting of an Inherited Thrombophilia and Atrial Septal Defect Sun, 27 Aug 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3159363/ We present a case of renal infarction in a 43-year-old female with history of stroke at age 14. She was found to be heterozygous for the prothrombin G20210A gene mutation. Loop monitoring revealed no atrial fibrillation. Transthoracic and transesophageal echocardiograms showed no thrombus. However, there was a small shunt due to an atrial septal defect (ASD). She was treated with warfarin and had device closure of her ASD. This was a suspected case of paradoxical embolism through an ASD leading to renal infarction. Siavash Piran and Sam Schulman Copyright © 2017 Siavash Piran and Sam Schulman. All rights reserved. Ponatinib as a Valid Alternative Strategy in Patients with Blast Crisis-Chronic Myeloid Leukemia Not Eligible for Allogeneic Stem Cells Transplantation and/or Conventional Chemotherapy: Report of a Case Mon, 14 Aug 2017 07:25:54 +0000 http://www.hindawi.com/journals/crihem/2017/6167345/ Currently, imatinib and dasatinib are the only tyrosine-kinase inhibitors approved in the US and Europe for the treatment of blast crisis of chronic myeloid leukemia (BC-CML) at diagnosis, while ponatinib is the only inhibitor used in patients bearing T315I mutation. Here we report the case of a 61-year-old man diagnosed with B-cell lymphoid BC-CML, initially treated with imatinib 800 mg day and then with dasatinib 140 mg day because of intolerance. A complete cytogenetic response (CCyR) was achieved at three months; however, three months later a relapse was observed, and the T315I mutation was detected. Ponatinib 45 mg once daily was then started together with a short course of chemotherapy. Bone marrow evaluation after six months of therapy showed the regaining of CCyR, together with the achievement of a deep molecular response. However, one year from ponatinib start the patient experienced a new disease relapse; he was effectively treated with ponatinib and chemotherapy once again, but in the meanwhile an ischemic stroke was detected. This case report confirms the high efficacy of ponatinib monotherapy in BC-CML patients, representing a valid option for non-allogeneic stem cells transplantation eligible cases and the only one available for those carrying the T315I mutation. Cristina Bucelli, Daniele Cattaneo, Valeria Ferla, Manuela Zappa, Caterina de Benedittis, Simona Soverini, and Alessandra Iurlo Copyright © 2017 Cristina Bucelli et al. All rights reserved. High-Grade B-Cell Neoplasm with Surface Light Chain Restriction and Tdt Coexpression Evolved in a MYC-Rearranged Diffuse Large B-Cell Lymphoma: A Dilemma in Classification Sun, 13 Aug 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/6891957/ According to World Health Organization (WHO) classification (2008), B-cell neoplasms are classified into precursor B-cell or a mature B-cell phenotype and this classification was also kept in the latest WHO revision (2016). We are reporting a male patient in his fifties, with tonsillar swelling diagnosed as diffuse large B-cell lymphoma (DLBCL), germinal center. He received 6 cycles of RCHOP and showed complete metabolic response. Two months later, he presented with severe CNS symptoms. Flow cytometry on bone marrow (BM) showed infiltration by CD10-positive Kappa-restricted B-cells with loss of CD20 and CD19, and downregulation of CD79b. Moreover, the malignant population showed Tdt expression. BM Cytogenetics revealed t(8;14)(q24;q32) within a complex karyotype. Retrospectively, MYC and Tdt immunostains performed on original diagnostic tissue and came negative for Tdt and positive for MYC. It has been rarely reported that mature B-cell neoplasms present with features of immaturity; however the significance of Tdt acquisition during disease course was not addressed before. What is unique in this case is that the emerging disease has acquired an immaturity marker while retaining some features of the original mature clone. No definitive WHO category would adopt high-grade neoplasms that exhibit significant overlapping features between mature and immature phenotypes. Dina Sameh Soliman, Ahmad Al-Sabbagh, Feryal Ibrahim, Ruba Y. Taha, Zafar Nawaz, Sarah Elkourashy, Abdulrazzaq Haider, Susanna Akiki, and Mohamed Yassin Copyright © 2017 Dina Sameh Soliman et al. All rights reserved. Decreased Rivaroxaban Levels in a Patient with Cerebral Vein Thrombosis Receiving Phenytoin Thu, 10 Aug 2017 06:29:24 +0000 http://www.hindawi.com/journals/crihem/2017/4760612/ Combined use of antiepileptic drugs and anticoagulants is common. We describe the first case documenting laboratory interaction between rivaroxaban and phenytoin. A 48-year-old woman was admitted to our hospital due to cerebral venous thrombosis, bilateral pulmonary embolism, and deep vein thrombosis. She came from a small town with difficult access to warfarin monitoring. She was receiving phenytoin 100 mg three times daily (t.i.d.) and started enoxaparin 60 mg twice daily (b.i.d.). An abdominal mass was diagnosed and removed by laparoscopy (gastrointestinal stromal tumor). On day 5, she was switched to rivaroxaban 15 mg b.i.d. First peak anti-Factor Xa was 70 ng/ml (reference value: 100–300 ng/ml). She was discharged on rivaroxaban 15 mg b.i.d. and phenytoin 100 mg t.i.d. A week later, anti-Xa levels were 90 ng/ml. Due to concerns about thrombosis progression, she was switched to dabigatran. During follow-up, she remained asymptomatic and thrombin time >180 s was measured several times along 3 months as surrogate for dabigatran activity. Phenytoin is a combined CYP3A4 and P-glycoprotein inducer, which might reduce rivaroxaban levels. Dabigatran is substrate of P-glycoprotein, meaning potential malabsorption. Despite unavailability of plasmatic dabigatran essays, our patient improved her symptoms without further symptomatic thromboembolism. Facing these interactions, either monitoring serum levels of anticoagulants or other therapeutic options should be considered. Ana F. Becerra, Tomas Amuchastegui, and Aldo H. Tabares Copyright © 2017 Ana F. Becerra et al. All rights reserved. Bone Marrow-Liver-Spleen Type of Large B-Cell Lymphoma Associated with Hemophagocytic Syndrome: A Rare Aggressive Extranodal Lymphoma Tue, 01 Aug 2017 08:10:54 +0000 http://www.hindawi.com/journals/crihem/2017/8496978/ Recently, an unusual subtype of large B-cell lymphoma (LBCL) with distinctive clinicopathologic features has been recognized; it is characterized by involvement of bone marrow with or without liver and/or spleen, but no lymph node or other extranodal sites, usually associated with fever, anemia, and hemophagocytic lymphohistiocytosis (HLH). Because of this distinctive clinical presentation, it has been designated “bone marrow-liver-spleen” (BLS) type of LBCL. To date there is only one series of 11 cases of BLS type of LBCL with detailed clinical, pathologic, and cytogenetic data. Herein, we describe a case of BLS type LBCL presenting with associated HLH in a 73-year-old female. The bone marrow core biopsy showed cytologically atypical large lymphoma cells present in a scattered interstitial distribution and hemophagocytosis and infrequent large lymphoma cells were seen in the bone marrow aspirate smears. Circulating lymphoma cells were not seen in the peripheral blood smears. The patient underwent treatment with chemotherapy (R-CHOP) but unfortunately passed away 2 months after initial presentation. BLS type of LBCL is a very rare and clinically aggressive lymphoma whose identification may be delayed by clinicians and hematopathologists due to its unusual clinical presentation and pathologic features. Kirill A. Lyapichev, Jennifer R. Chapman, Oleksii Iakymenko, Offiong F. Ikpatt, Uygar Teomete, Sandra Patricia Sanchez, and Francisco Vega Copyright © 2017 Kirill A. Lyapichev et al. All rights reserved. Sequential Kinase Inhibition (Idelalisib/Ibrutinib) Induces Clinical Remission in B-Cell Prolymphocytic Leukemia Harboring a 17p Deletion Thu, 27 Jul 2017 08:32:07 +0000 http://www.hindawi.com/journals/crihem/2017/8563218/ B-cell prolymphocytic leukemia (B-PLL) is a rare lymphoid neoplasm with an aggressive clinical course. Treatment strategies for B-PLL remain to be established, and, until recently, alemtuzumab was the only effective therapeutic option in patients harboring 17p deletions. Herein, we describe, for the first time, a case of B-cell prolymphocytic leukemia harboring a 17p deletion in a 48-year-old man that was successfully treated sequentially with idelalisib-rituximab/ibrutinib followed by allogeneic hematopoietic stem cell transplant (allo-HSCT). After 5 months of therapy with idelalisib-rituximab, clinical remission was achieved, but the development of severe diarrhea led to its discontinuation. Subsequently, the patient was treated for 2 months with ibrutinib and the quality of the response was maintained with no severe adverse effects reported. A reduced-intensity conditioning allo-HSCT from a HLA-matched unrelated donor was performed, and, thereafter, the patient has been in complete remission for 10 months now. In conclusion, given the poor prognosis of B-PLL and the lack of effective treatment modalities, the findings here suggest that both ibrutinib and idelalisib should be considered as upfront therapy of B-PLL and as a bridge to allo-HSCT. H. Coelho, M. Badior, and T. Melo Copyright © 2017 H. Coelho et al. All rights reserved. Spontaneous Complete Remission in a Patient with Acute Myeloid Leukemia and Severe Sepsis Mon, 24 Jul 2017 07:24:30 +0000 http://www.hindawi.com/journals/crihem/2017/9593750/ Without treatment, acute myeloid leukemia (AML) is almost always fatal. Spontaneous remission of AML is a rare phenomenon and usually with a short duration. The exact mechanisms are unknown. However, its association with infection and blood transfusions has been described. We report a 53-year-old male who presented with severe sepsis and who was diagnosed with AML (M4). He has experienced complete spontaneous remission with relatively long duration. To the best of our knowledge, it is the first case of spontaneous remission described in Iran. Rambod Mozafari, Mahsa Moeinian, and Ali Asadollahi-Amin Copyright © 2017 Rambod Mozafari et al. All rights reserved. Immune Thrombocytopenia and JAK2V617F Positive Essential Thrombocythemia: Literature Review and Case Report Thu, 20 Jul 2017 08:14:41 +0000 http://www.hindawi.com/journals/crihem/2017/3725089/ We present the case where immune thrombocytopenia (ITP) and essential thrombocythemia (ET) sequentially appeared in the space of twenty-one years of follow-up. Impaired platelet production is present in both diseases, but clinical presentation and treatment are different. On the basis of this case history a possible role of autoimmunity as a predisposing factor to myeloproliferation has been discussed. M. A. Sobas, T. Wróbel, K. Zduniak, M. Podolak-Dawidziak, J. Rybka, M. Biedroń, M. Sawicki, J. Dybko, and K. Kuliczkowski Copyright © 2017 M. A. Sobas et al. All rights reserved. Large Granular Lymphocytic Leukemia: A Report of Response to Rituximab Tue, 18 Jul 2017 07:16:16 +0000 http://www.hindawi.com/journals/crihem/2017/7506542/ Large granular lymphocytic (LGL) leukemia is a rare form of low grade leukemia characterized by large cytotoxic T cells or natural killer cells on morphological examination. Immunosuppressive therapy is employed as first-line therapy. Treatment options in refractory cases include the anti-CD52 antibody alemtuzumab and purine analogues. We report a rare case that responded to the anti-CD20 monoclonal antibody rituximab. A 77-year-old female presented with complaints of fatigue, fever, and chills of 3 months’ duration. A CBC showed that pancytopenia with an absolute neutrophil count (ANC) was 0. Peripheral blood flow cytometry detected increased number of T cell large granular lymphocytes and T cell receptor rearrangement study detected a clonal T cell population. Bone marrow biopsy showed peripheral T cell lymphoma, most consistent with T-large granulocytic leukemia. The patient was treated with prednisone and oral cyclophosphamide for four months with no response. Thereafter, she received four weekly infusions of rituximab with improvement in her blood counts. A response to rituximab in refractory cases such as ours has been reported and may guide us towards exploring other immune-based therapeutics in this rare disease. Uroosa Ibrahim, Sara Parylo, Shiksha Kedia, Shafinaz Hussein, and Jean Paul Atallah Copyright © 2017 Uroosa Ibrahim et al. All rights reserved. Ibrutinib Treatment of Mantle Cell Lymphoma Relapsing at Central Nervous System: A Case Report and Literature Review Sun, 16 Jul 2017 07:36:54 +0000 http://www.hindawi.com/journals/crihem/2017/9583257/ Mantle cell lymphoma (MCL) accounts for about 5% of all lymphomas. Its clinical and histological features are heterogeneous. After a frequently good initial response, the disease generally and repeatedly relapses and finally the outcome is poor. Particularly severe is the prognosis of the rare occurrence of CNSi (Central Nervous System involvement). Ibrutinib, an oral inhibitor of Bruton tyrosine kinase (BTK), has shown strong activity in relapsing patients with Chronic Lymphocytic Leukemia (CLL) and MCL. Few reports are available about treatment with ibrutinib of patients presenting CNSi by lymphoproliferative diseases (LPD). In all of them, ibrutinib, at the dosage between 420 and 560 mg/day, showed an impressive effectiveness. Here we describe a case of MCL with CNS relapse showing an excellent response to ibrutinib administered at the unusual dose of 280 mg/day because of concomitant treatment of cardiological disease. Donato Mannina and Barbara Loteta Copyright © 2017 Donato Mannina and Barbara Loteta. All rights reserved. Incidental Discovery of Multiorgan Extramedullary Plasmacytomas in the Setting of Newly Diagnosed Multiple Myeloma and Delayed Hemolytic Transfusion Reaction Thu, 06 Jul 2017 06:48:53 +0000 http://www.hindawi.com/journals/crihem/2017/4531858/ Extramedullary plasmacytomas (EMPs) are defined by the presence of clonal plasma cell proliferation outside of the bone marrow, portending an overall poor prognosis. This case highlights extramedullary plasmacytomas as an unusual presenting manifestation of multiple myeloma. Through incidental discovery during a delayed hemolytic transfusion reaction workup, EMPs were found in the liver, spleen, and possibly the lung. Though rare at presentation, this case emphasizes that the presence of EMPs should be considered at the outset as it not only impacts the treatment regimen for such patients but also considerably affects prognosis. Joselle Cook, Steven Song, Anthony Ventimiglia, and Carol Luhrs Copyright © 2017 Joselle Cook et al. All rights reserved. An Atypical Presentation of Chronic Atrophic Gastritis: Hemolytic Anemia and Mesenteric Panniculitis Mon, 03 Jul 2017 06:52:27 +0000 http://www.hindawi.com/journals/crihem/2017/5498034/ Microangiopathic hemolytic anemia (MAHA) requires an aggressive approach since primary thrombotic microangiopathy syndromes such as thrombotic thrombocytopenic purpura (TTP) can progress rapidly to a fatal outcome. Differential diagnosis can be challenging even for an experienced hematologist. We present a case of a 52-year-old male who presented with symptoms of mesenteric panniculitis and showed signs of MAHA. His condition was attributed to severe vitamin B12 deficiency secondary to chronic atrophic gastritis and initiation of appropriate therapy was met with complete resolution of symptoms and normalization of hematologic parameters. Zurab Azmaiparashvili, Vinicius M. Jorge, and Catiele Antunes Copyright © 2017 Zurab Azmaiparashvili et al. All rights reserved. Prostatic-Like Syndrome in a Woman with Chronic Lymphocytic Leukemia: Sequential Kinase Inhibitor Therapy Sun, 02 Jul 2017 09:03:12 +0000 http://www.hindawi.com/journals/crihem/2017/3869020/ Chronic lymphocytic leukemia (CLL) is an incurable lymphoproliferative disorder with a heterogeneous genetic and clinical course. Two kinase inhibitors, ibrutinib and idelalisib, have demonstrated achievement of complete and durable remissions in relapse/refractory genetically unselected CLL patients. We present a case of relapsed CLL with extensive disease and hourglass deformity of urinary bladder as a result of the compression of two extraperitoneal paravesical soft tissue bulky masses, with excellent response to sequential kinase inhibitor therapy. Diego Velasco-Rodríguez, Miguel Piris-Villaespesa, Carmen Soteras, Ana Vallés, José Antonio García-Marco, and José Antonio García-Vela Copyright © 2017 Diego Velasco-Rodríguez et al. All rights reserved. Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the Literature Tue, 13 Jun 2017 09:36:42 +0000 http://www.hindawi.com/journals/crihem/2017/7185604/ Bone marrow necrosis (BMN) is a rare clinical entity that was first described in an autopsy of a sickle cell disease (SCD) patient and is defined as ill-defined necrotic cells in an amorphous eosinophilic background with preservation of cortical bone. The pathophysiology of BMN is not well known; however, occlusion of the bone marrow microcirculation with subsequent hypoxia and cell injury has been thought to be common underlying features. Malignancy has been identified to be the primary cause in 90% of the cases whereas SCD was found in only 2%. In this report we present an unusual case of SCD with late onset of the disease whose initial presentation was extensive BMN. The patient was not known previously to have SCD, when suddenly she presented with severe cytopenias and marked elevation in serum lactate dehydrogenase (LDH). Bone marrow examination was done to exclude bone marrow infiltration, and BMN with dilated marrow sinuses full of irreversibly sickled cells were the unexpected findings. Patients with a mild SCD phenotype are at high risk of BMN. Thus, a high index of suspicion must be borne in mind, particularly in an area of high SCD prevalence, to recognize and prevent this catastrophic complication. Sameera A. Alsafwani, Abdulwahed Al-Saeed, and Rehab Bukhamsin Copyright © 2017 Sameera A. Alsafwani et al. All rights reserved. Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom’s Macroglobulinemia Tue, 13 Jun 2017 07:43:01 +0000 http://www.hindawi.com/journals/crihem/2017/8795213/ A 73-year-old male with long-standing Waldenstrom’s macroglobulinemia complicated with systemic amyloidosis presented with a witnessed syncopal episode. He had complaints of orthostatic dizziness and palpitations for few months. Orthostatic hypotension and peripheral neuropathy were demonstrated on physical examination. EKG, 24-hour Holter monitoring, and 2D echocardiogram were unremarkable. MRI of the brain ruled out stroke. Patients with amyloidosis can develop cardiovascular disease through amyloid cardiomyopathy, small vessel disease, conduction defects, pericardial effusion, or autonomic denervation. After ruling out other life-threatening causes, Ewing’s battery of tests was done to rule out cardiac autonomic neuropathy. Two heart rate tests and one blood pressure test were abnormal which indicated severe cardiac autonomic neuropathy. Cardiac autonomic neuropathy can mask symptoms of acute coronary syndrome and hence early diagnosis using the simple bedside maneuver is beneficial. The test is also important for prognostication. Absence of augmentation of cardiac output from inadequate autonomic stimulation will lead to postural hypotension, exercise intolerance, and tachycardia. There may be no change in heart rate with Valsalva or deep breathing both of which increase parasympathetic tone. As the condition progresses, it may result in cardiac denervation which can result in silent myocardial infarction, syncope, and sudden death. Aasems Jacob, Rishi Raj, and Warren Walkow Copyright © 2017 Aasems Jacob et al. All rights reserved. Vertebral Artery Thrombosis in Chronic Idiopathic Thrombocytopenic Purpura Sun, 11 Jun 2017 08:29:44 +0000 http://www.hindawi.com/journals/crihem/2017/3184346/ Introduction. Immune thrombocytopenic purpura (ITP) is an autoimmune hematological disorder that causes decreased production and destruction of platelets leading to thrombocytopenia. Although thrombocytopenia usually causes hemorrhagic problems, thrombotic events like strokes, although rare, can still occur. Management of thrombotic events in patients with ITP differs from that of patients with normal platelet count function and count. Case Description. A 32-year-old female with a history of ITP presented with ischemic stroke. The patient was treated in the hospital with IV immunoglobulin, discharged to a rehabilitation facility, and had complete resolution of symptoms when examined at a follow-up visit 3 months later. Conclusion. Although stroke in patients with ITP is very rare due to thrombocytopenia, it has been reported in several other published cases and is likely associated with increased platelet microparticle levels, a byproduct of platelet destruction. While usage of antiplatelet therapy in such patients is debated, immunosuppression therapy has been the mainstay treatment in all published cases. Zakaria Hindi, Nirmal Onteddu, Christopher A. Ching, and Abdallah A. Khaled Copyright © 2017 Zakaria Hindi et al. All rights reserved. Sustained Remission in Patients with Primary Immune Thrombocytopenia after Romiplostim Tapering and Discontinuation: A Case Series in Real Life Management in Spain Sun, 11 Jun 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/4109605/ Romiplostim, a thrombopoietin-receptor agonist (TPO-ra), is a highly effective option in primary immune thrombocytopenia (ITP), with 80–90% of patients achieving platelet responses after few weeks of treatment. The evidence showing remissions, that is, sustained platelet counts after romiplostim discontinuation, in patients with ITP refractory to immunosuppressive therapy is steadily increasing. However, there is a lack of guidelines or recommendations addressing how and when to taper romiplostim in clinical practice in patients maintaining elevated and stable platelet counts. Furthermore, given the high heterogeneity of ITP patients, no associated predictive factors have been currently identified. Here, we present 4 representative clinical cases of the daily clinical practice in Spain comprising newly diagnosed, persistent, and both splenectomized and nonsplenectomized chronic ITP patients treated with romiplostim, achieving and maintaining clinical remission (platelet count ≥ 50 109/L for 24 consecutive weeks in the absence of any treatment for ITP) after treatment tapering and discontinuation, without observed safety concerns. Prospective studies identifying clinical and biological predictive factors of sustained response are warranted. María-Eva Mingot-Castellano, Carlos Grande-García, David Valcárcel-Ferreiras, Clara Conill-Cortés, and Loreto de Olivar-Oliver Copyright © 2017 María-Eva Mingot-Castellano et al. All rights reserved. Dermatomyositis Associated with Myelofibrosis following Polycythemia Vera Sun, 04 Jun 2017 06:44:07 +0000 http://www.hindawi.com/journals/crihem/2017/9091612/ Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (PV) who developed proximal muscle weakness, dysphagia, and rash. He was found to have elevated creatinine kinase and skin biopsy was consistent with DM. Due to persistent pancytopenia a bone marrow biopsy was performed and showed postpolycythemic myelofibrosis. To our knowledge, this is the first case reported of this unique association. Naomi Fei and Sarah Sofka Copyright © 2017 Naomi Fei and Sarah Sofka. All rights reserved. Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma Mon, 29 May 2017 08:04:40 +0000 http://www.hindawi.com/journals/crihem/2017/9862620/ Acquired von Willebrand syndrome (AVWS) is an increasingly recognized entity with numerous potential underlying etiologies. Most commonly implicated are lymphoproliferative, myeloproliferative, cardiovascular, and autoimmune disorders. Unlike inherited von Willebrand disease (vWD), AVWS tends to present at an older age and without a family history of vWD. Treatment is directed at the underlying etiology if one is uncovered, as well as treatment and prevention of bleeding. Here, we present a rare case of AVWS secondary to Waldenström macroglobulinemia which went unrecognized for several years but resolved promptly with treatment. The potential mechanisms of AVWS secondary to monoclonal gammopathies are discussed as well as strategies to treat and prevent bleeding in these patients. Zachary Wolfe and Bradley Lash Copyright © 2017 Zachary Wolfe and Bradley Lash. All rights reserved. A Rare Case of Paraneoplastic Aortitis Associated with Chronic Myelomonocytic Leukemia Thu, 25 May 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3091973/ Aortitis is a broad term describing inflammation of the aorta. The most common causes of aortitis are the large-vessel vasculitides giant cell arteritis and Takayasu arteritis. Other etiologies include aortitis associated with other autoimmune disorders, infectious causes, and paraneoplastic and idiopathic cases. We describe a rare case of a large-vessel arteritis occurring in association with chronic myelomonocytic leukemia (CMML). A 68-year-old female with recent diagnosis of CMML presented to our office for evaluation of abnormal chest computed tomography (CT) that showed inflammation surrounding the entirety of thoracic and abdominal aorta, consistent with aortitis. In the absence of other evident causes of large-vessel vasculitis, we attributed this finding to a paraneoplastic autoimmune phenomenon and started treatment with systemic glucocorticoids. This rare case emphasizes the need to recognize autoimmune complications in CMML and treat the inflammation along with the primary malignancy promptly. Sylwia Sasinowska, Pamela Traisak, Michael McCormack, and Hala Eid Copyright © 2017 Sylwia Sasinowska et al. All rights reserved. Thrombotic Microangiopathy Secondary to Intravenous Abuse of Opana® ER Thu, 18 May 2017 06:19:41 +0000 http://www.hindawi.com/journals/crihem/2017/1623907/ Opana ER (oxymorphone) is an opioid drug available throughout the United States, and intravenous abuse of the crushed oral formulation has been associated with drug-induced thrombotic microangiopathy. In this abstract, we describe two young patients who lived together and used Opana ER intravenously. Both presented with microangiopathic hemolytic anemia that mimicked thrombotic thrombocytopenic purpura (TTP). Treating this condition poses a clinical challenge, as it is difficult to distinguish it from TTP. The role for plasma exchange is not clear but can be used while awaiting the results of the ADAMTS-13 activity, but ultimately supportive care with drug discontinuation is the recommended therapy of choice. Patients should be counseled against Opana ER’s intravenous use, and users should be offered drug rehabilitation therapy. Kamia Thakur, Vaibhav Agrawal, Ashley Kass, Lauren M. Dimarino, R. Patrick Dorion, and Joseph Vadakara Copyright © 2017 Kamia Thakur et al. All rights reserved. Molecular Profiling: A Case of ZBTB16-RARA Acute Promyelocytic Leukemia Wed, 26 Apr 2017 06:57:37 +0000 http://www.hindawi.com/journals/crihem/2017/7657393/ Several variant RARA translocations have been reported in acute promyelocytic leukemia (APL) of which the t(11;17)(q23;q21), which results in a ZBTB16-RARA fusion, is the most widely identified and is largely resistant to therapy with all-trans retinoic acid (ATRA). The clinical course together with the cytogenetic and molecular characterization of a case of ATRA-unresponsive ZBTB16-RARA APL is described. Additional mutations potentially cooperating with the translocation fusion product in leukemogenesis have been hitherto unreported in ZBTB16-RARA APL and were sought by application of a next-generation sequencing approach to detect those recurrently found in myeloid malignancies. This technique identified a solitary, low level mutation in the CEBPA gene. Molecular profiling of additional mutations may provide a platform to individualise therapeutic management in patients with this rare form of APL. Stephen E. Langabeer, Lisa Preston, Johanna Kelly, Matt Goodyer, Ezzat Elhassadi, and Amjad Hayat Copyright © 2017 Stephen E. Langabeer et al. All rights reserved. Coexistence of Antiphospholipid Syndrome and Heparin-Induced Thrombocytopenia in a Patient with Recurrent Venous Thromboembolism Wed, 26 Apr 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3423548/ Heparin-induced thrombocytopenia (HIT) is a prothrombotic adverse drug reaction in which heparin forms complexes with platelet factor 4 forming neoantigens that are recognized by autoantibodies. Antiphospholipid syndrome (APS) is similar to HIT in that it is mediated by autoantibodies that are also prothrombotic. We present a case of rare coexistence of antiphospholipid antibody syndrome and heparin-induced thrombocytopenia. Samuel Adediran and Nicole Agostino Copyright © 2017 Samuel Adediran and Nicole Agostino. All rights reserved. A Rare Case of Angioimmunoblastic T-Cell Lymphoma with Epstein-Barr Virus-Negative Reed-Sternberg-Like B-Cells, Chylous Ascites, and Chylothorax Wed, 12 Apr 2017 07:57:38 +0000 http://www.hindawi.com/journals/crihem/2017/1279525/ Angioimmunoblastic T-cell lymphoma is a rare non-Hodgkin lymphoma with dismal prognosis. The median age of presentation ranges from 62 to 69 years with generalized lymphadenopathy, B symptoms, and hepatosplenomegaly as the most prevalent symptoms. The combination of B-cell and T-cell proliferations is common in AITL and the B-cell component may resemble Reed-Sternberg-like B-cells. Epstein-Barr virus is estimated to be present in 80–95% of AITL biopsies. Only a handful of EBV-negative AITL cases with EBV-negative RS-like B-cells have been reported over the last decade. We present a rare case of EBV-negative AITL with chylous ascites and chylothorax. Microscopic and immunohistochemical analysis revealed the presence of EBV-negative Reed-Sternberg-like B-cells in the tumor. Mathijs Willemsen, Arne W. J. H. Dielis, Iryna V. Samarska, Ad Koster, and Arienne M. van Marion Copyright © 2017 Mathijs Willemsen et al. All rights reserved. EBV-Negative Monomorphic B-Cell Posttransplant Lymphoproliferative Disorder with Marked Morphologic Pleomorphism and Pathogenic Mutations in ASXL1, BCOR, CDKN2A, NF1, and TP53 Mon, 10 Apr 2017 06:15:19 +0000 http://www.hindawi.com/journals/crihem/2017/5083463/ Posttransplant lymphoproliferative disorders (PTLDs) are a diverse group of lymphoid or plasmacytic proliferations frequently driven by Epstein-Barr virus (EBV). EBV-negative PTLDs appear to represent a distinct entity. This report describes an unusual case of a 33-year-old woman that developed a monomorphic EBV-negative PTLD consistent with diffuse large B-cell lymphoma (DLBCL) 13 years after heart-lung transplant. Histological examination revealed marked pleomorphism of the malignant cells including nodular areas reminiscent of classical Hodgkin lymphoma (cHL) with abundant large, bizarre Hodgkin-like cells. By immunostaining, the malignant cells were immunoreactive for CD45, CD20, CD79a, PAX5, BCL6, MUM1, and p53 and negative for CD15, CD30, latent membrane protein 1 (LMP1), and EBV-encoded RNA (EBER). Flow cytometry demonstrated lambda light chain restricted CD5 and CD10 negative B-cells. Fluorescence in situ hybridization studies (FISH) were negative for cMYC, BCL2, and BCL6 rearrangements but showed deletion of TP53 and monosomy of chromosome 17. Next-generation sequencing studies (NGS) revealed numerous genetic alterations including 6 pathogenic mutations in ASXL1, BCOR, CDKN2A, NF1, and TP53(x2) genes and 30 variants of unknown significance (VOUS) in ABL1, ASXL1, ATM, BCOR, BCORL1, BRNIP3, CDH2, CDKN2A, DNMT3A, ETV6, EZH2, FBXW7, KIT, NF1, RUNX1, SETPB1, SF1, SMC1A, STAG2, TET2, TP53, and U2AF2. Agata M. Bogusz Copyright © 2017 Agata M. Bogusz. All rights reserved.