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    417 results match your criteria Case reports in hematology[Journal]

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    Unusual Presentation of a Small-Cell Variant of Anaplastic Large-Cell Lymphoma Case: When a Septic Picture Is Not Sepsis.
    Case Rep Hematol 2017 24;2017:7292148. Epub 2017 Sep 24.
    Department of Hematology and Oncology, William Beaumont Hospital, Royal Oak, MI, USA.
    We report a case of a small-cell variant of anaplastic large-cell lymphoma, with an unusual clinical presentation mimicking sepsis and a fulminant clinic course, in a 48-year-old Caucasian female. In this report, we discuss the diagnostic challenge, histopathologic features, and unique cytogenetic features of this case, in order to raise awareness of this rare presentation and emphasize the importance of meticulous peripheral smear examination and early bone marrow evaluation. Read More

    Upper Limb Deep Vein Thrombosis in Patient with Hemophilia A and Heterozygosity for Prothrombin G20210A: A Case Report and Review of the Literature.
    Case Rep Hematol 2017 25;2017:7290945. Epub 2017 Sep 25.
    Department of Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
    Deep vein thrombosis (DVT) is a rare disease in patients with hemophilia A. We report a case of 22-year-old male with severe hemophilia A who presented to the emergency room with 5-day history of right arm pain that was attributed initially to bleeding event. In the absence of external signs of bleeding or hematoma and normal hemoglobin level, we suspected an underlying DVT. Read More

    Paroxysmal Nocturnal Hemoglobinuria in Pregnancy: A Dilemma in Treatment and Thromboprophylaxis.
    Case Rep Hematol 2017 24;2017:7289126. Epub 2017 Sep 24.
    Department of Medicine, Division of Hematology & Oncology, University of Florida, Gainesville, FL, USA.
    Paroxysmal nocturnal hemoglobinuria (PNH) is a hematologic disorder characterized by an acquired somatic mutation in the phosphatidylinositol glycan class A gene which leads to a higher risk for increased venous and arterial thrombosis. Current treatment for PNH includes eculizumab. Pregnant patients who have PNH have higher risk for thrombosis and hemorrhage with both pregnancy and their underlying PNH. Read More

    γδ T-Cell Acute Lymphoblastic Leukemia/Lymphoma: Discussion of Two Pediatric Cases and Its Distinction from Other Mature γδ T-Cell Malignancies.
    Case Rep Hematol 2017 24;2017:5873015. Epub 2017 Sep 24.
    Department of Pathology and Translational Pathobiology, LSU Health Shreveport, Shreveport, LA, USA.
    Gamma delta (γδ) T-cell antigen receptor (TCR) expression and its related T-cell differentiation are not commonly reported in T-cell acute lymphoblastic leukemia/lymphoma (T-ALL). Here we report two pediatric T-ALL cases and present their clinical features, histology, immunophenotypes, cytogenetics, and molecular diagnostic findings. The first patient is a two-year-old girl with leukocytosis, circulating lymphoblasts, and a cryptic insertion of a short-arm segment at 10p12 into the long-arm segment of 11q23 resulting in an MLL and AF10 fusion transcript, which may be the first reported in γδ T-ALL. Read More

    Chronic Myeloid Leukemia with an e6a2 BCR-ABL1 Fusion Transcript: Cooperating Mutations at Blast Crisis and Molecular Monitoring.
    Case Rep Hematol 2017 16;2017:9071702. Epub 2017 Oct 16.
    Cancer Molecular Diagnostics, St. James's Hospital, Dublin 8, Ireland.
    A minority of chronic myeloid leukemia patients (CML) express a variety of atypical BCR-ABL1 fusion variants and, of these, the e6a2 BCR-ABL1 fusion is generally associated with an aggressive disease course. Progression of CML to blast crisis is associated with acquisition of additional somatic mutations yet these events have not been elucidated in patients with the e6a2 BCR-ABL1 genotype. Moreover, molecular monitoring is only sporadically performed in CML patients with atypical BCR-ABL1 fusion transcripts due to lack of consensus approaches or standardization. Read More

    PAX5-Negative Classical Hodgkin Lymphoma: A Case Report of a Rare Entity and Review of the Literature.
    Case Rep Hematol 2017 4;2017:7531729. Epub 2017 Oct 4.
    Department of Pathology and Laboratory Medicine, University of California, Davis, Davis, CA, USA.
    Classical Hodgkin lymphoma (CHL) is recognized as a B-cell neoplasm arising from germinal center or postgerminal center B-cells. The hallmark of CHL is the presence of CD30 (+) Hodgkin and Reed-Sternberg (HRS) cells with dim expression of PAX5. Nearly all of the HRS cells are positive for PAX5. Read More

    HbS-Sicilian (δβ)(0)-Thalassemia: A Rare Variant of Sickle Cell.
    Case Rep Hematol 2017 17;2017:9265396. Epub 2017 Sep 17.
    Cleveland Clinic Lerner College of Medicine (CCLCM), Case Western University, 9500 Euclid Avenue, Cleveland, OH 44195, USA.
    Sickle cell disease (SCD) is caused by a mutation in the sixth codon of the β-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. Read More

    Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia.
    Case Rep Hematol 2017 28;2017:8174207. Epub 2017 Sep 28.
    Department of Pathology and Laboratory Medicine, Brown University Alpert Medical School, Providence, RI, USA.
    Hemoglobin (Hb) Sherwood Forest is a rare high-affinity hemoglobin first described in 1977, arising from an Arg to Thr substitution at codon 104 of the beta chain. This hemoglobin variant has been identified in few individuals and has been associated with a compensatory erythrocytosis in the homozygous state. Prior scarce case reports have noted that heterozygotes for this variant are phenotypically normal. Read More

    Plasmablastic Lymphoma: Case Report of Prolonged Survival of an Advanced Human Immunodeficiency Patient and Literature Review.
    Case Rep Hematol 2017 24;2017:9561013. Epub 2017 Sep 24.
    Division of Hematology Oncology, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.
    Clinical Practice Points. Plasmablastic lymphoma (PBL) is a rare and highly aggressive variant of diffuse large B cell lymphoma with median survival of advanced stage patients varying between 6 and 15 months in previous reports. We report here a human immunodeficiency virus-infected patient surviving over 12 years following treatment for advanced PBL with EPOCH chemotherapy and intrathecal therapy. Read More

    Polycythaemia Secondary to Hormone Replacement Therapy with Tibolone.
    Case Rep Hematol 2017 27;2017:3476349. Epub 2017 Sep 27.
    Haematology Department, The Canberra Hospital, Canberra, ACT, Australia.
    We present the case report of a patient with severe polycythaemia associated with tibolone. In our 65-year-old postmenopausal patient who initially presented with haemoglobin 203 g/L [115-160] and haematocrit 0.63 [0. Read More

    Posttransplant Lymphoproliferative Disorder in a Patient with Worsening Ascites after Liver Transplantation.
    Case Rep Hematol 2017 11;2017:7247438. Epub 2017 Sep 11.
    McGovern Medical School, Department of Gastroenterology, Transplant and Advanced Hepatology, The University of Texas Health Science Center at Houston, 6431 Fannin St., Suite MSB 1.134, Houston, TX 77030, USA.
    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. Read More

    Primary Cutaneous Follicle Centre Lymphoma with Hodgkin and Reed-Sternberg Like Cells: A Case Report and Review of the Literature.
    Case Rep Hematol 2017 26;2017:9549428. Epub 2017 Sep 26.
    Department of Pathology and Laboratory Medicine, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, RI, USA.
    An elderly woman with a complex medical history presented with a left forearm mass that slowly developed for several months. The excisional biopsy of this skin mass was remarkable for involvement by a follicle centre cell derived lymphoma with a nodular and diffuse pattern associated with a subset of scattered Hodgkin and Reed-Sternberg like cells. Fluorescence in situ hybridization studies did not detect the presence of IgH-bcl2 fusion transcript, and molecular studies were negative for immunoglobulin heavy chain gene rearrangements and EBV DNA sequences. Read More

    A Case of Rituximab-Induced Necrotizing Fasciitis and a Review of the Literature.
    Case Rep Hematol 2017 26;2017:6971027. Epub 2017 Sep 26.
    Department of Medicine, Reading Hospital, West Reading, PA, USA.
    Necrotizing fasciitis is a fulminant soft tissue infection characterized by rapid progression and high mortality. Rituximab is a generally well-tolerated immunosuppresive medication used for B-cell malignancies and some rheumatological disorders. We report a case of a 69-year-old male with chronic lymphocytic leukemia who suffered necrotizing fasciitis of his left lower extremity secondary to Clostridium septicum 7 weeks after treatment with rituximab. Read More

    An Unsuspected Finding of t(9;22): A Rare Case of Philadelphia Chromosome-Positive B-Lymphoblastic Lymphoma.
    Case Rep Hematol 2017 18;2017:2413587. Epub 2017 Sep 18.
    Department of Leukemia, UT MD Anderson Cancer Center, Houston, TX, USA.
    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. Read More

    Cutaneous Vasculitis: An Unusual Presentation of a Biclonal Nodal Plasma Cell Dyscrasia.
    Case Rep Hematol 2017 13;2017:8152610. Epub 2017 Sep 13.
    Haematology Department, University Hospital Waterford, Regional Cancer Center South East, University College Cork, Cork, Ireland.
    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. Read More

    Immune-Mediated Autonomic Neuropathies following Allogeneic Stem Cell Transplantation in Acute Myeloid Leukemia.
    Case Rep Hematol 2017 27;2017:6803804. Epub 2017 Aug 27.
    Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
    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. Read More

    Acute Unilateral Renal Infarction in the Setting of an Inherited Thrombophilia and Atrial Septal Defect.
    Case Rep Hematol 2017 27;2017:3159363. Epub 2017 Aug 27.
    Department of Medicine, Division of Hematology and Thromboembolism and Thrombosis and Atherosclerosis Research Institute, McMaster University, Hamilton, ON, Canada.
    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. Read More

    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.
    Case Rep Hematol 2017 14;2017:6167345. Epub 2017 Aug 14.
    Hematology Division, IRCCS Ca' Granda-Maggiore Policlinico Hospital Foundation and University of Milan, Milan, Italy.
    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. Read More

    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.
    Case Rep Hematol 2017 13;2017:6891957. Epub 2017 Aug 13.
    Department of Hematology and Medical Oncology, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.
    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. Read More

    Decreased Rivaroxaban Levels in a Patient with Cerebral Vein Thrombosis Receiving Phenytoin.
    Case Rep Hematol 2017 10;2017:4760612. Epub 2017 Aug 10.
    Vascular Medicine and Thrombosis Service, Hospital Privado Universitario de Cordoba, Cordoba, Argentina.
    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. Read More

    Bone Marrow-Liver-Spleen Type of Large B-Cell Lymphoma Associated with Hemophagocytic Syndrome: A Rare Aggressive Extranodal Lymphoma.
    Case Rep Hematol 2017 1;2017:8496978. Epub 2017 Aug 1.
    Division of Hematopathology, Department of Pathology and Laboratory Medicine, University of Miami and Sylvester Comprehensive Cancer Center, Miami, FL, USA.
    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. Read More

    Sequential Kinase Inhibition (Idelalisib/Ibrutinib) Induces Clinical Remission in B-Cell Prolymphocytic Leukemia Harboring a 17p Deletion.
    Case Rep Hematol 2017 27;2017:8563218. Epub 2017 Jul 27.
    Serviço de Hematologia, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova Gaia, Portugal.
    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). Read More

    Spontaneous Complete Remission in a Patient with Acute Myeloid Leukemia and Severe Sepsis.
    Case Rep Hematol 2017 24;2017:9593750. Epub 2017 Jul 24.
    Department of Infectious Disease, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
    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. Read More

    Immune Thrombocytopenia and JAK2V617F Positive Essential Thrombocythemia: Literature Review and Case Report.
    Case Rep Hematol 2017 20;2017:3725089. Epub 2017 Jul 20.
    Department of Haematology, Blood Neoplasms and Bone Marrow Transplantation, Medical University of Wrocław, Wrocław, Poland.
    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. Read More

    Large Granular Lymphocytic Leukemia: A Report of Response to Rituximab.
    Case Rep Hematol 2017 18;2017:7506542. Epub 2017 Jul 18.
    Department of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA.
    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. Read More

    Ibrutinib Treatment of Mantle Cell Lymphoma Relapsing at Central Nervous System: A Case Report and Literature Review.
    Case Rep Hematol 2017 16;2017:9583257. Epub 2017 Jul 16.
    Hematology Unit, Papardo Hospital, c/da Papardo, 98158 Messina, Italy.
    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. Read More

    Incidental Discovery of Multiorgan Extramedullary Plasmacytomas in the Setting of Newly Diagnosed Multiple Myeloma and Delayed Hemolytic Transfusion Reaction.
    Case Rep Hematol 2017 6;2017:4531858. Epub 2017 Jul 6.
    Division of Hematology and Oncology, SUNY Downstate Medical Center, Brooklyn, NY 11203, USA.
    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. Read More

    An Atypical Presentation of Chronic Atrophic Gastritis: Hemolytic Anemia and Mesenteric Panniculitis.
    Case Rep Hematol 2017 3;2017:5498034. Epub 2017 Jul 3.
    Albert Einstein Medical Center, Philadelphia, PA, USA.
    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. Read More

    Prostatic-Like Syndrome in a Woman with Chronic Lymphocytic Leukemia: Sequential Kinase Inhibitor Therapy.
    Case Rep Hematol 2017 2;2017:3869020. Epub 2017 Jul 2.
    Department of Hematology, Hospital Universitario de Getafe, Madrid, Spain.
    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. Read More

    Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma.
    Case Rep Hematol 2017 29;2017:9862620. Epub 2017 May 29.
    Department of Hematology and Oncology, Guthrie Clinic/Robert Packer Hospital, Sayre, PA, USA.
    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. Read More

    Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom's Macroglobulinemia.
    Case Rep Hematol 2017 13;2017:8795213. Epub 2017 Jun 13.
    Department of Internal Medicine, Monmouth Medical Center, 300 2nd Ave, Long Branch, NJ 07740, USA.
    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. Read More

    Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia-A Case Report and Review of the Literature.
    Case Rep Hematol 2017 13;2017:7185604. Epub 2017 Jun 13.
    Dammam Regional Laboratory and Blood Bank (DRL), Dammam, Saudi Arabia.
    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%. Read More

    Sustained Remission in Patients with Primary Immune Thrombocytopenia after Romiplostim Tapering and Discontinuation: A Case Series in Real Life Management in Spain.
    Case Rep Hematol 2017 11;2017:4109605. Epub 2017 Jun 11.
    Amgen S.A., Moll de Barcelona, Edifici Sud, Planta 7, 08039 Barcelona, Spain.
    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. Read More

    Vertebral Artery Thrombosis in Chronic Idiopathic Thrombocytopenic Purpura.
    Case Rep Hematol 2017 11;2017:3184346. Epub 2017 Jun 11.
    School of Medicine, University of Jordan, Amman, Jordan.
    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. Read More

    Dermatomyositis Associated with Myelofibrosis following Polycythemia Vera.
    Case Rep Hematol 2017 4;2017:9091612. Epub 2017 Jun 4.
    Department of Internal Medicine, West Virginia University Hospital, 1 Medical Center Dr., Morgantown, WV 26505, USA.
    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. Read More

    A Rare Case of Paraneoplastic Aortitis Associated with Chronic Myelomonocytic Leukemia.
    Case Rep Hematol 2017 25;2017:3091973. Epub 2017 May 25.
    Division of Rheumatology, Cooper University Hospital, Camden, NJ, USA.
    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. Read More

    Thrombotic Microangiopathy Secondary to Intravenous Abuse of Opana® ER.
    Case Rep Hematol 2017 18;2017:1623907. Epub 2017 May 18.
    Geisinger Medical Center, Danville, PA, USA.
    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). Read More

    Coexistence of Antiphospholipid Syndrome and Heparin-Induced Thrombocytopenia in a Patient with Recurrent Venous Thromboembolism.
    Case Rep Hematol 2017 26;2017:3423548. Epub 2017 Apr 26.
    Lehigh Valley Health Network, Departments of Hematology and Oncology and Medicine, Allentown, PA, USA.
    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. Read More

    Molecular Profiling: A Case of ZBTB16-RARA Acute Promyelocytic Leukemia.
    Case Rep Hematol 2017 26;2017:7657393. Epub 2017 Apr 26.
    Department of Haematology, Galway University Hospital, Galway, Ireland.
    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. Read More

    EBV-Negative Monomorphic B-Cell Posttransplant Lymphoproliferative Disorder with Marked Morphologic Pleomorphism and Pathogenic Mutations in ASXL1, BCOR, CDKN2A, NF1, and TP53.
    Case Rep Hematol 2017 10;2017:5083463. Epub 2017 Apr 10.
    Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA 19104-4283, USA.
    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. Read More

    A Rare Case of Angioimmunoblastic T-Cell Lymphoma with Epstein-Barr Virus-Negative Reed-Sternberg-Like B-Cells, Chylous Ascites, and Chylothorax.
    Case Rep Hematol 2017 12;2017:1279525. Epub 2017 Apr 12.
    Department of Pathology, VieCuri Medical Centre, 5912 BL Venlo, Netherlands.
    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. Read More

    Acquired Thrombotic Thrombocytopenic Purpura in a Patient with Pernicious Anemia.
    Case Rep Hematol 2017 4;2017:1923607. Epub 2017 Apr 4.
    Interfaith Medical Center, Brooklyn, NY, USA.
    Introduction. Acquired thrombotic thrombocytopenic purpura (TTP) has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported. Read More

    Successful Long-Term Use of Eltrombopag in a Patient with Refractory Severe Thrombocytopenia Associated with Chronic Lymphocytic Leukemia That Allowed Oral Anticoagulant Treatment for Severe Cardiomyopathy.
    Case Rep Hematol 2017 2;2017:9538920. Epub 2017 Apr 2.
    Division of Hematology, Principe de Asturias University Hospital, Madrid, Spain.
    Autoimmune cytopenias (AICs) are frequently associated with chronic lymphocytic leukemia (CLL). The most common of these AICs is autoimmune hemolytic anemia (AIHA); the second most is immune thrombocytopenia (ITP). Here, we report on a patient with CLL-associated ITP, with thrombocytopenia refractory to corticosteroids and intravenous immunoglobulins, in which continuous oral treatment with Eltrombopag allowed initiation and maintenance of an oral anticoagulation treatment with Acenocoumarol that was indicated because of a severe arrhythmogenic cardiomyopathy. Read More

    Extensive Intracardiac and Deep Venous Thromboses in a Young Woman with Heparin-Induced Thrombocytopenia and May-Thurner Syndrome.
    Case Rep Hematol 2017 30;2017:8530476. Epub 2017 Mar 30.
    Montefiore Heart & Vascular Care Center, Albert Einstein College of Medicine, Bronx, NY, USA.
    A 38-year-old woman with a history of recurrent deep venous thromboses (DVTs) on chronic anticoagulation presented with acute left leg swelling. The patient was diagnosed with an acute left lower extremity (LLE) DVT in the setting of May-Thurner syndrome for which treatment with unfractionated heparin was started. Her hospital course was complicated by a new diagnosis of heparin-induced thrombocytopenia (HIT), with an incidental discovery of a large tricuspid valve mobile mass on a transthoracic echocardiogram (TTE). Read More

    Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature.
    Case Rep Hematol 2017 28;2017:2057019. Epub 2017 Mar 28.
    Hemostasis and Thrombosis Unit, Division of Hematology, Cliniques Universitaires Saint-Luc, 1200 Brussels, Belgium.
    Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Read More

    Acute Myeloid Leukemia with Basophilic Differentiation Transformed from Myelodysplastic Syndrome.
    Case Rep Hematol 2017 27;2017:4695491. Epub 2017 Mar 27.
    Department of Hematology and Clinical Immunology, Nishi-Kobe Medical Center, Hyogo, Japan.
    Myelodysplastic syndrome (MDS) terminally transforms to acute myeloid leukemia (AML) or bone marrow failure syndrome, but acute myeloid leukemia with basophilic differentiation has been rarely reported. An 81-year-old man was referred to our department for further examination of intermittent fever and normocytic anemia during immunosuppressive treatment. Chromosomal analysis showed additional abnormalities involving chromosome 7. Read More

    Celiac Disease Presenting with Immune Thrombocytopenic Purpura.
    Case Rep Hematol 2017 23;2017:6341321. Epub 2017 Mar 23.
    Pediatric Gastroenterology, Denizli State Hospital, Denizli, Turkey.
    Celiac disease (CD) is an immunological disorder. Clinical manifestations occur as a result of intestinal mucosa damage and malabsorption. CD is also associated with extraintestinal manifestations and autoimmune disorders. Read More

    Extramedullary Relapse in a CML Patient after Allogeneic Stem Cell Transplantation.
    Case Rep Hematol 2017 21;2017:6350267. Epub 2017 Mar 21.
    Hematology Department, Ege University Hospital, İzmir, Turkey.
    Myeloid or granulocytic sarcoma (GS) is a tumoral lesion consisting of immature granulocytic cells. It is a rare entity during the course of CML patients especially after allogeneic stem cell transplantation (SCT). Relapse without bone marrow involvement is much rarer. Read More

    A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Extensively Studied by Flow Cytometry and Immunohistochemistry.
    Case Rep Hematol 2017 20;2017:4984951. Epub 2017 Mar 20.
    Division of Hematology, Niguarda Ca' Granda Hospital, Milan, Italy.
    Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive clinical course and poor prognosis. Diagnosis is based on detection of CD4(+) CD56(+), CD123(high), TCL-1(+), and blood dendritic cell antigen-2/CD303(+) blasts, together with the absence of lineage specific antigens on tumour cells. In this report we present a case of BPDCN presenting with extramedullary and bone marrow involvement, extensively studied by flow cytometry and immunohistochemistry, who achieved complete remission after acute lymphoblastic leukemia like chemotherapy and allogeneic hematopoietic stem cell transplantation. Read More

    Remission of Severe, Relapsed, and Refractory TTP after Multiple Cycles of Bortezomib.
    Case Rep Hematol 2017 6;2017:9681832. Epub 2017 Mar 6.
    Leukemia Service, Department of Medicine, Roswell Park Cancer Institute, Buffalo, NY, USA.
    Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by autoantibodies against a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Uncleaved von Willebrand factor (VWF) multimers accumulate and bind to platelets which causes spontaneous microthrombi ultimately causing microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemia. Plasma exchange (PEX) with or without steroids constitutes standard first-line therapy with rituximab typically reserved for refractory cases. Read More

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