Dataset Information

Progressive Heart Failure and Death as the Initial Manifestation of NK/T-Cell Lymphoma: A Case Report and Literature Review.

ABSTRACT: Natural killer/T-cell (NK/T-cell) lymphoma is a rare-type non-Hodgkin lymphoma derived from NK cells or cytotoxic T cells. Here, we present a case of a 40-year-old woman who experienced quick-developed global heart failure and then was diagnosed with NK/T-cell lymphoma through lymphoid biopsy. Neither transthoracic echocardiography nor any radiological images detected a mass in her heart or pericardium. Elevated plasma troponin level and diffused patchy areas of gadolinium late enhancement on cardiac magnetic resonance were compatible with myocarditis. Considering the persistently elevated cytokine level, systemic inflammation symptoms, acute respiratory distress syndrome, and cardiac dysfunction, a cytokine storm secondary to NK/T-cell lymphoma was considered. Due to the refractory malignant arrhythmia, the patient died soon after being admitted to our hospital.

SUBMITTER: Zhang Z

PROVIDER: S-EPMC8264061 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature

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Progressive Heart Failure and Death as the Initial Manifestation of NK/T-Cell Lymphoma: A Case Report and Literature Review.

Zhang Ziyu Z Wang Shuai S Liang Qingchun Q Peng Daoquan D

Frontiers in cardiovascular medicine 20210624

Natural killer/T-cell (NK/T-cell) lymphoma is a rare-type non-Hodgkin lymphoma derived from NK cells or cytotoxic T cells. Here, we present a case of a 40-year-old woman who experienced quick-developed global heart failure and then was diagnosed with NK/T-cell lymphoma through lymphoid biopsy. Neither transthoracic echocardiography nor any radiological images detected a mass in her heart or pericardium. Elevated plasma troponin level and diffused patchy areas of gadolinium late enhancement on ca ...[more]

PMID: 34250042

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Project description:IntroductionMegalosplenia in newly diagnosed multiple myeloma (MM) is extremely rare, posing diagnostic and therapeutic challenges due to its unusual location and clinical manifestations and lack of optimal therapeutic strategies.Case presentationA 65-year-old female who was previously healthy presented with a history of ecchymosis on her right leg accompanied by progressive fatigue for 2 weeks. She was admitted to our center in July 2019 due to thrombocytopenia. The patient presented with megalosplenia, anemia, monoclonal protein (λ-light chain type) in the serum and urine, and 45.6% malignant plasma cells in the bone marrow. Splenectomy was performed due to persistent splenomegaly after 3 cycles of the bortezomib plus dexamethasone regimen, and immunohistochemistry results indicated λ-plasmacytoma of the spleen. The same cytogenetic and molecular abnormalities, including t(14;16), 14q32 amplification, 16q32 amplification, 20q12 amplification, and a novel CYLD gene mutation, were identified using fluorescence in situ hybridization and next-generation sequencing in both bone marrow and spleen samples. Therefore, a diagnosis of MM (λ-light chain type, DS III, ISS III, R-ISS III, high-risk) with spleen infiltration was proposed. The patient did not achieve remission after induction treatment with bortezomib plus lenalidomide and dexamethasone or salvage therapy with daratumumab plus ixazomib and dexamethasone. However, she ultimately did achieve very good partial remission with a regimen of bendamustine plus lenalidomide and dexamethasone. Unfortunately, she died of pneumonia associated with chemotherapy.ConclusionTo our knowledge, only 8 cases of spleen plasmacytoma at MM diagnosis have been described previously. Extramedullary myeloma patients with spleen involvement at diagnosis are younger and that the condition is usually accompanied by splenic rupture with aggressive clinical features and poor prognosis. Further studies are needed to explore pathogenesis and effective therapies to prolong the survival of such patients.

Dataset Information

Progressive Heart Failure and Death as the Initial Manifestation of NK/T-Cell Lymphoma: A Case Report and Literature Review.

Publications

Progressive Heart Failure and Death as the Initial Manifestation of NK/T-Cell Lymphoma: A Case Report and Literature Review.

Similar Datasets

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