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Case Reports in Hematology

Faaria Gowani, Bradley Gehrs, Teresa Scordino
Drug-induced hypersensitivity syndrome (DIHS; also known as drug reaction with eosinophilia and systemic symptoms, or DRESS) is a rare, potentially life-threatening condition that typically presents 2-8 weeks after drug exposure with fever, rash, organ dysfunction, and lymphadenopathy. Here, we describe the case of an 18-year-old African American female who presented with cervical lymphadenopathy, fevers, and a macular rash. A PET scan showed diffuse hypermetabolic lymphadenopathy suggestive of lymphoma, with involvement of the spleen and kidneys...
2018: Case Reports in Hematology
Sebastiano Cicco, Antonio G Solimando, Patrizia Leone, Stefano Battaglia, Roberto Ria, Angelo Vacca, Vito Racanelli
Primary systemic amyloidosis is not easily diagnosed. The immunoglobulin deposits are usually localized in the kidney, heart, and liver. We describe an unusual case of a patient suffering from a pericardial amyloidoma with internal calcifications and air bubbles that compressed the right ventricle and shifted the heart to the left. Since the patient was in shock, urgent pericardiotomy was performed. This site showed PET uptake. A monoclonal component was present. On these findings, differential diagnoses included multiple myeloma and atypical pericardial tuberculosis, whereas a periumbilical fat tissue biopsy demonstrated amyloidosis...
2018: Case Reports in Hematology
Panayotis Kaloyannidis, Eshrak Al Shaibani, Miral Mashhour, Mohammed Gamil, Ioannis Apostolidis, Hani Al Hashmi, Khalid Ahmed Al Anazi
The programmed cell death protein-1 (PD-1) inhibitor nivolumab has been recently approved as an effective and safe treatment for patients with refractory/relapsed Hodgkin's lymphomas. Dermatological adverse events, mainly skin rash, have been reported in 1-5% of patients. We describe a case of de novo psoriasis vulgaris (PsV), diagnosed after nivolumab treatment for refractory Hodgkin's lymphoma. After administration of 6 cycles, skin lesions appeared in the right tibia, forearms, and dorsum of hands, and biopsy confirmed the diagnosis of PsV...
2018: Case Reports in Hematology
Kosuke Miki, Naoshi Obara, Kenichi Makishima, Tatsuhiro Sakamoto, Manabu Kusakabe, Takayasu Kato, Naoki Kurita, Hidekazu Nishikii, Yasuhisa Yokoyama, Mamiko Sakata-Yanagimoto, Yuichi Hasegawa, Shigeru Chiba
We report the case of a 76-year-old man who was diagnosed as having chronic myeloid leukemia (CML) with p190 BCR-ABL while receiving treatment for symptomatic multiple myeloma (MM). The diagnosis of MM was based on the presence of serum M-protein, abnormal plasma cells in the bone marrow, and lytic bone lesions. The patient achieved a partial response to lenalidomide and dexamethasone treatment. However, 2 years after the diagnosis of MM, the patient developed leukocytosis with granulocytosis, anemia, and thrombocytopenia...
2018: Case Reports in Hematology
Manuel R Espinoza-Gutarra, Sherri L Cervantez, Zohra Nooruddin
CNS involvement by CLL is a rare occurrence, usually happening in the context of a transformation to a more aggressive lymphoma in what is known as Richter's transformation. We report a patient with active CLL who developed confusion and was found to have West Nile encephalitis that initially mimicked CNS involvement by CLL. The patient recovered with supportive treatment and later restarted ibrutinib therapy. This case illustrates the importance of maintaining a broad differential among cancer patients with new onset confusion as well as that of questioning malignant infiltration of CNS when there is concomitant active CNS infection...
2018: Case Reports in Hematology
David Ferreira, Royston Ponraj, Adrian Yeung, Jillian de Malmanche
Pure red cell aplasia is an uncommon cause of anaemia rarely associated with thymoma. A combination of immunosuppressive therapy and thymectomy offers a potential cure. Thymectomy alone rarely results in anaemia resolution. A seventy-three-year-old male with Klinefelter syndrome presented with progressively increasing shortness of breath and anaemia. Serological testing supported primary bone marrow pathology, and a bone marrow biopsy was performed. A pure red cell aplasia was seen on bone marrow examination, and computed tomography of the chest demonstrated a thymoma...
2018: Case Reports in Hematology
Florentina Matea, Salem Alowami, Michael Bonert, Monalisa Sur, Yaron Shargall, Asghar H Naqvi
Intravascular large B cell lymphoma (IVLBCL) is a rare type of extranodal diffuse large B-cell lymphoma. Patients typically present with nonspecific findings, particularly bizarre neurologic symptoms, fever, and skin lesions. IVLBCL with primary lung presentation is very rare and difficult to diagnose. The authors report a case of a 75-year-old male who presented with neurological symptoms and showed diffuse pulmonary ground glass opacities on computed tomography scan (CT scan). Surgical lung biopsy was performed...
2018: Case Reports in Hematology
Khaled Algashaamy, Yaohong Tan, Nicolas Mackrides, Alvaro Alencar, Jing-Hong Peng, Joseph Rosenblatt, Juan P Alderuccio, Izidore S Lossos, Francisco Vega, Jennifer Chapman
Prolymphocytic transformation is a concept usually applied in the context of chronic lymphocytic leukemia/small lymphocytic lymphoma to describe the presence of a high percentage of prolymphocytes in peripheral blood (usually more than 55%). Prolymphocytic transformation has also been reported in mantle cell lymphoma (MCL) but only rarely in splenic marginal zone lymphoma (SMZL). We present two splenic B-cell lymphomas presenting in the leukemic phase and with increased prolymphocytes, both classified as SMZL with prolymphocytic transformation...
2018: Case Reports in Hematology
Andrew Chu, Thu Thu Aung, Minni Shreya Arumugam, Mauricio Danckers, Mohi Mitiek, Jonathan Leslie
Factor V Leiden (FVL) is an autosomal dominant condition resulting in thrombophilia. Factor V normally acts as a cofactor for prothrombinase, helping cleave prothrombin to thrombin. A single point mutation in it disrupts factor V, making it unreceptive to protein C and increasing the risk of thrombosis. FVL mutation associated with right heart thrombus is a rare entity. Right heart thrombus or right heart thrombus-in-transit is associated with high mortality. We present a 51-year-old male with a past medical history of FVL homozygous mutation and recurrent blood clots, who has failed multiple different oral anticoagulants...
2018: Case Reports in Hematology
Mohamed Hassanein, Lilit Karapetyan, Afshan Khan, Supratik Rayamajhi
Sarcoidosis is a systemic inflammatory disease characterized by the presence of noncaseating granulomas in different organs. Sarcoidosis associated with monoclonal gammopathy of undetermined significance (MGUS) is a rare finding with only 10 cases reported to date. We describe a 79-year-old male patient who presented with dry mouth for 4 months. Lip biopsy done prior to admission showed nonnecrotizing epithelioid cell granulomas. On admission, laboratory analysis was significant for elevated calcium, decreased parathyroid hormone, increased erythrocyte sedimentation rate, undetectable parathyroid hormone-related peptide (PTHrp), mildly decreased 25-hydroxyvitamin D, elevated 1,25-dihydroxyvitamin D, elevated angiotensin converting enzyme, and positive Bence Jones protein in the urine...
2018: Case Reports in Hematology
Jonas Juul Hansen, Hans Beier Ommen, Lars Christian Gormsen, Francesco Annibale d'Amore, Peter Martin Hjørnet Kamper
We report a case presenting with recurrent episodes of severe hypothermia preceding the diagnosis of Hodgkin lymphoma. The episodes of hypothermia were accompanied by other symptoms of autonomic dysfunction, mainly hypotension, which could be caused by autonomic neuropathy as part of a paraneoplastic syndrome. In comparison with previous reports describing an association between the presence of hypothermia and an adverse outcome, the present patient has responded well to lymphoma-specific treatment and is currently in an ongoing complete remission...
2018: Case Reports in Hematology
Robert Stuver, Alec Petersen, Thomas A Guerrero-Garcia, Ursula Matulonis, Paul Richardson, Prabhsimranjot Singh
Multiple myeloma is the most common plasma cell dyscrasia and causes 2% of all cancer deaths in Western countries. Ovarian carcinosarcomas are very rare gynecological malignancies and account for only 1-2% of all ovarian tumors. In this case, we report a 67-year-old woman with known relapsed ovarian carcinosarcoma who presented with headache and neck pain. She was found to have new lytic lesions in the cranial and thoracic regions. While these lesions were assumed to be metastases, a diligent approach detected an M-spike on serum protein electrophoresis and a monoclonal gammopathy with immunoglobulin G lambda monoclonal immunoglobulin on immunofixation...
2018: Case Reports in Hematology
Håkon Reikvam, Jørn Skavland, Stein-Erik Gullaksen, Randi Hovland, Tobias Gedde-Dahl, Øystein Bruserud, Bjørn Tore Gjertsen
Chronic myeloid leukemia (CML) is a myeloproliferative disorder in which neoplastic cells exhibit the Philadelphia chromosome and the related oncoprotein BCR-ABL1 . Allogeneic stem cell transplantation (allo-SCT) was considered the first-line treatment for CML, before the introduction of tyrosine kinase inhibitors (TKIs). However, patients are at risk for relapse years after transplantation. We present a patient who relapsed 25 years after allo-SCT for chronic phase CML. Polymerase chain reaction (PCR) detected gradually evaluated levels of BCR-ABL1 transcripts, eventually leading to the diagnosis of relapsed disease...
2018: Case Reports in Hematology
Madiha Iqbal, Rabia Saleem, Salman Ahmed, Prachi Jani, Salvador Alvarez, Han W Tun
Phlegmonous gastritis is an uncommon acute bacterial infection of the stomach that carries a fatal prognosis in spite of the advent of antibiotics. A high index of suspicion is required in patients with risk factors. An immunocompromised state is identified as one of the most important risk factors. We hereby report a case of successful antimicrobial treatment of phlegmonous gastritis in a patient who was receiving intensive chemotherapy for acute myelogenous leukemia. We have also carried out a review of literature over the past ten years...
2018: Case Reports in Hematology
Vadim R Gorodetskiy, Wolfram Klapper, Natalya A Probatova, Vladimir I Vasilyev, Elena V Rozhnova
We present an exceptionally rare case of co-occurrence of Rosai-Dorfman disease (RDD) and nodal marginal zone lymphoma (NMZL) in a 60-year-old Caucasian female with a 20-year course of Sjögren's syndrome (SS). In response to treatment for lymphoma, the patient presented a short positive response, followed by a rapid progression of the disease accompanied by the development of the peripheral facial nerve palsy. We failed to detect Epstein-Barr virus (EBV) in the NMZL/RDD sample by EBV-encoded RNA (EBER) in situ hybridization but identified genomic DNA of EBV by polymerase chain reaction...
2018: Case Reports in Hematology
Shyam Ravisankar, Yair Levy, Maya Shah
Acute myeloid leukemia (AML) can present with extramedullary involvement known as myeloid sarcoma (MS). We present the case of a young woman who was diagnosed with AML and MS in bilateral orbits, brain, omentum, and retroperitoneum. She was treated with induction chemotherapy. Low-dose radiation was given to the orbits due to visual symptoms which resulted in complete response. The use of radiation therapy in orbital MS has not been studied extensively, and low dose may be adequate to achieve complete remission (CR) in selected patients...
2018: Case Reports in Hematology
Eric Granowicz, Kiyon Chung
Cardiac disease is a well-known complication of antiphospholipid syndrome (APS), with many patients presenting with valvular thickening or vegetations, referred to as Libman-Sacks endocarditis (LSE). Because cases of APS with cardiac involvement are relatively rare, paucity of large clinical trials studying this complication has made management challenging. In the absence of acute heart failure and embolic events, a medical approach is usually selected, consisting of anticoagulation and possibly corticosteroids when another underlying autoimmune disease is present...
2018: Case Reports in Hematology
Amy G Starr, Sushma R Jonna, Joeffrey J Chahine, Bhaskar V Kallakury, Chaitra S Ujjani
Lymphadenopathy in chronic myeloid leukemia (CML) is usually due to extramedullary involvement with accelerated or blast phases of the disease. The occurrence of non-Hodgkin lymphoma (NHL) as a synchronous malignancy with CML is rare. We report a case of a 73-year-old male who presented with dyspnea and right-sided lower extremity edema in the setting of leukocytosis. Bone marrow evaluation indicated a chronic phase chronic myeloid leukemia (CML), confirmed by molecular testing. Imaging of the chest for persistent dyspnea revealed supraclavicular and mediastinal lymphadenopathy...
2018: Case Reports in Hematology
Nigel P Murray, Lorena Muñoz, Simona Minzer, Marco Antonio Lopez
Nonvalvular atrial fibrillation (AF) is a common age-related arrthymia and a leading cause of stroke in the elderly; with an aging hemophilia population, the number of patients developing AF is increasing. There are no controlled trials on thromboprophylaxis in this group of patients, only consensus opinion was based on small case reports. We present a female patient, carrier for hemophilia and with clinically moderately severe hemophilia who developed FA. We discuss the literature with respect to this group of patients and current recommendations for thromboprophylaxis...
2018: 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
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