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Case Reports in Hematology

Daniel S O'Neil, Mark A Francescone, Karen Khan, Alobeid Bachir, Owen A O'Connor, Ahmed Sawas
Bing-Neel syndrome is a rare manifestation of Waldenström macroglobulinemia characterized by lymphoplasmacytic cells' infiltration into the central nervous system. We present a case of a 74-year-old patient with a known diagnosis of Waldenström macroglobulinemia and newly depressed consciousness. Flow cytology of his cerebral spinal fluid demonstrated a lambda light chain-restricted population of B-cells consistent with a CD5+ CD10+ B-cell lymphoma. Magnetic resonance imaging suggested involvement of the left optic nerve sheath and the bilateral orbital and parietal parenchyma and leptomeninges...
2018: Case Reports in Hematology
Alexandros Makis, Zoi Kanta, Dimitrios Kalogeropoulos, Nikoloaos Chaliasos
Autoimmune hemolytic anemia (AIHA) is a relatively uncommon hematological entity in children and sometimes is characterized by a severe course requiring more than one line course therapy. Treatment decisions depend on the severity and chronicity of the anemia and the characteristics of the autoantibodies. Immunosuppression with corticosteroids is the first-line treatment, especially in warm-reactive AIHA. Refractory cases are treated with immunosuppressive drugs, cytotoxic agents, androgens, or splenectomy, with various side effects and questionable efficacy...
2018: Case Reports in Hematology
Leena T Rahmat, Anna Nguyen, Haifaa Abdulhaq, Sonam Prakash, Aaron C Logan, Gabriel N Mannis
Long-term disease-free survival in adults with T-cell acute lymphoblastic leukemia (T-ALL) remains poor, particularly after relapse, with few available salvage options. Preclinical data suggest that inhibition of the antiapoptotic protein BCL-2 (B-cell lymphoma 2) either alone or in combination with other agents, may be a unique therapeutic approach for the treatment of T-ALL. We present a case of a young male with T-ALL, relapsed after allogeneic hematopoietic stem cell transplant, who achieved a second complete remission following salvage therapy with combined venetoclax and decitabine...
2018: Case Reports in Hematology
Fiorella Villano, Adriana Peixoto, Eloísa Riva, Carina Di Matteo, Lilián Díaz
Digital ischemia is associated with atherosclerotic, thromboembolic, or connective tissue diseases. Less often, it can be related to malignancy. Paraneoplastic vascular acrosyndromes (Raynaud's syndrome, acrocianosis, and acronecrosis) are associated with adenocarcinoma and less frequently with hematological malignancies. We report the case of a 45-year-old male, smoker, with a 10-day history of pain, cyanosis, and progressive digital necrosis in both hands. In the previous four months, he noticed painless mass in the right axillary gap, drenching night sweats, and weight loss...
2018: Case Reports in Hematology
Tamim Alsuliman, Maifa Belghoul, Bachra Choufi
Nowadays, mantle cell lymphoma is considered to have one of the worst prognostic profiles among lymphoid malignancies. Mantle cell lymphoma rarely affects the central nervous system (CNS) as it represents about 0.9% of diagnosis and 4% among recurrent cases. Here, we present the case of a 69-year-old male patient who was diagnosed with mantle cell lymphoma in 2006. The patient relapsed three times, without affecting the CNS, then was treated accordingly, and achieved complete remission three times. Four years after his last complete remission, upon receiving his last dose of treatment, the medical team noted a rapid worsening of the patient's neurological status followed by a deep coma state causing MCL neurological recurrence by exclusion diagnosis...
2018: Case Reports in Hematology
I Ramasamy, Z Rudzki
Gamma heavy chain disease ( γ -HCD) is a rare lymphoproliferative disorder characterised by the production of a truncated immunoglobulin heavy chain. Fewer than 200 cases have been reported in the literature. In some cases, γ -HCD occurs with other lymphoid neoplasms. This study reports clinical, biochemical, haematological, and histological findings in two cases of γ -HCD. We describe newer biochemical diagnostic tools (HevyLite measurement, capillary electrophoresis, and immunotyping) that can aid in the characterisation of γ -HCD...
2018: Case Reports in Hematology
Fares Darawshy, Arieh Ben-Yehuda, Karine Atlan, Deborah Rund
Background: Chronic lymphocytic lymphoma (CLL) can be associated with several malignancies, but rarely with myelofibrosis. Only isolated case reports in the literature described the association between CLL and primary myelofibrosis (PMF) in the same patient. Objectives: We describe a case of CLL characterized by the development of PMF and a review of literature. Methods: We describe an 86-year-old female diagnosed as having CLL and followed by the development of splenomegaly and progressively rising LDH levels 27 months later...
2018: Case Reports in Hematology
Karan Seegobin, Bharatsinh Gharia, Satish Maharaj, Lara Zuberi
Essential thrombocythemia (ET) occurring with breast cancer is uncommon; the therapeutic approach varies and poses a challenge. A 65-year-old female presented to us after being diagnosed with hormone positive, HER2-negative infiltrating ductal carcinoma. She had a platelet count of 600 thou/cu mm. Her JAK2 mutation was positive. Bone marrow biopsy showed increased megakaryocytes. She was diagnosed with ET in the setting of breast cancer. She underwent breast conservation surgery after which aspirin was resumed...
2018: Case Reports in Hematology
Ensi Voshtina, Huiya Huang, Renju Raj, Ehab Atallah
Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in Western countries. A common first-line therapy offered to qualifying patients includes ibrutinib, an oral covalent inhibitor of Bruton's tyrosine kinase. Treatment of CLL with ibrutinib therapy is generally well tolerated; however, serious opportunistic infections are being reported in patients treated with ibrutinib. In this report, we present a patient with CLL on ibrutinib therapy who developed rapidly declining neurological status concerning for the central nervous system (CNS) process related to his immunocompromised status...
2018: Case Reports in Hematology
John Patrick O'Neill, Fiona Quinn, Anita Dowling, Jan Walker, Triona Hayes, Brian Bird, Richard Flavin
A composite lymphoma is the rare simultaneous occurrence of two or more distinct lymphomas within a single tissue or organ. Herein, we describe a case of a 51-year-old man presenting with a history of lower limb rash, fatigue, and bulky abdominopelvic lymphadenopathy. An excisional left iliac lymph node biopsy was notable for the composite presence of two distinct lymphoid neoplasms, nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), and follicular lymphoma (FL). Multiplex PCR and FISH analyses failed to demonstrate a t(14;18)(q32;q21) translocation in either composite lymphoma component...
2018: Case Reports in Hematology
Lauren Mendelson, Emily Hsu, Hojune Chung, Andrew Hsu
Primary cardiac lymphoma (PCL) is a rare disease entity that can present with severe cardiac and cardioembolic symptoms. We present a 79-year-old male with history of polymalgia rheumatica on chronic prednisone who presented with a two-week history of progressively worsening dyspnea, cough, and a 10 pound weight loss. Transthoracic echocardiogram (TTE) and computed tomography (CT) of the chest showed a large mediastinal mass with invasion of the pericardium. A biopsy of an abdominal soft-tissue mass confirmed the diagnosis of PCL...
2018: Case Reports in Hematology
William A Hammond, Pooja Advani, Rhett P Ketterling, Daniel Van Dyke, James M Foran, Liuyan Jiang
Updated WHO criteria define mixed phenotype acute leukemia (MPAL) with more stringent diagnostic criteria than the formerly described entity biphenotypic acute leukemia (BAL). The changes in diagnostic criteria influence management by assigning weight to aberrantly expressed markers and minimizing expression of myeloid markers other than myeloperoxidase (MPO), potentially foregoing consolidative allogeneic transplant for an otherwise "favorable" lymphoid phenotypic leukemia. We present a case of MPO-negative, myeloid antigen-positive acute lymphoblastic leukemia who progressed with refractory phenotypic acute myeloid leukemia while receiving lymphoid-directed therapy and discuss concerns raised by the adoption of the new, more stringent diagnostic criteria for BAL...
2018: Case Reports in Hematology
Zarir Ahmed, Martin Schoen, Nabeel Rajeh
Myasthenia gravis is a very rare manifestation of graft versus host disease after stem cell transplants. Herein, we describe a case of new-onset myasthenia gravis after a stem cell transplant 34 months ago in a patient with myelodysplastic syndrome.
2018: Case Reports in Hematology
Dhauna Karam, Sean Swiatkowski, Mamata Ravipati, Bharat Agrawal
Aggressive systemic mastocytosis (ASM) is characterized by mast cell accumulation in systemic organs. Though ASM may be associated with other hematological disorders, the association with pure red cell aplasia (PRCA) is rare and has not been reported. Pure red cell aplasia (PRCA) is a syndrome, characterized by normochromic normocytic anemia, reticulocytopenia, and severe erythroid hypoplasia. The myeloid and megakaryocytic cell lines usually remain normal. Here, we report an unusual case of ASM, presenting in association with PRCA and the management challenges...
2018: Case Reports in Hematology
Hanyin Wang, Hande Tuncer
The value of combination therapy for refractory ITP is not well defined. We present the case of a 29-year-old male with severe ITP refractory to initial standard therapy including steroids, IVIG, and subsequent splenectomy, who was treated with the combination therapy of rituximab, romiplostim, and mycophenolate and eventually developed thrombocytosis requiring plateletpheresis. Our case highlights the importance of the need to understand predictors of response to standard upfront treatment of acute ITP.
2018: Case Reports in Hematology
Suheil Albert Atallah-Yunes, Myat Han Soe
Drug-induced thrombotic microangiopathies (DTMAs) are increasingly being recognized as an important category of thrombotic microangiopathies (TMAs). Cancer therapeutic agents including proteasome inhibitors (PIs) are among the most common medications reported to cause DTMA. PIs could cause DTMA either by an immune mechanism or dose-dependent/cumulative toxicity. Eleven cases of DTMA have been reported with bortezomib and carfilzomib. To the best of our knowledge, only one case of DTMA has been reported with ixazomib due to an immune-mediated mechanism...
2018: Case Reports in Hematology
Umer Syed, Kramer J Wahlberg, Daniel R Douce, Julian R Sprague
A 76-year-old male with metastatic renal carcinoma on day 24 of pazopanib was admitted with complaints of emesis, confusion, and hematuria. Laboratory testing showed acute kidney injury, hyperbilirubinemia, and thrombocytopenia. Scattered schistocytes were seen on peripheral smear, and he was diagnosed with thrombotic microangiopathy (TMA). He was started on daily, one-volume plasma exchange with rapid improvement in thrombocytopenia. ADAMTS13 activity returned as undetectably low with no inhibitor detected...
2018: Case Reports in Hematology
Kenji Miki, Takashi Maruki, Shinsaku Imashuku
Background: Spur cell anemia (SCA) is a cause of hemolytic anemia in patients with alcoholic liver cirrhosis. Because dyslipidemia is related to the development of spur cells, SCA was previously treated with plasmapheresis. Case Report: A 52-year-old Japanese man with SCA associated with alcoholic liver cirrhosis (Child-Pugh C) underwent two rounds of plasmapheresis. Clinical features and serum lipid concentrations were compared before and after plasmapheresis. Although indirect hyperbilirubinemia and SCA persisted after plasmapheresis, reticulocyte counts significantly decreased from 22...
2018: Case Reports in Hematology
Clara Chamba, Hamisa Iddy, Erius Tebuka, Furahini Tluway, Elisha Osati, Neema Budodi, Collins Meda, Mbonea Yonazi, Anna Schuh, Lucio Luzzatto, Julie Makani
Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD) with blood transfusion an integral part in its management. Red cell exchange (RCE) transfusion is usually regarded as preferable to top-up transfusion, because it reduces the proportion of Hemoglobin (Hb) S while at the same time avoiding circulatory overload. Despite its obvious benefits, RCE is underutilized, particularly in low-resource settings which may be due to scarcity of blood products and of expertise in carrying out exchange transfusion...
2018: Case Reports in Hematology
Takanori Fukuta, Takayuki Tanaka, Yoshinori Hashimoto, Hiromi Omura
While cases of multiple myeloma (MM) with metastatic calcification have been reported, the mechanisms for this calcification have yet to be explained. We observed a case of MM in a patient with end-stage renal failure who developed vascular and pulmonary calcification. A 51-year-old male was diagnosed with Bence-Jones type MM and required maintenance hemodialysis. He was treated with bortezomib-dexamethasone, vincristine-doxorubicin-dexamethasone, the M2 protocol, and lenalidomide-dexamethasone (Rd) therapy...
2018: Case Reports in Hematology
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