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Rapid treatment of leukostasis in leukemic mantle cell lymphoma using therapeutic leukapheresis: a case report.

ABSTRACT: We describe a case of severe leukocytosis caused by leukemic mantle cell lymphoma (MCL), complicated by leukostasis with myocardial infarction in which leukapheresis was used in the initial management. A 73-year-old male presented to the emergency department because of fatigue and thoracic pain. Blood count revealed 630 × 10(9)/L WBC (white blood cells). The electrocardiogram showed ST-elevation with an increase of troponin and creatinine kinase. The diagnosis was ST-elevation myocardial infarction (STEMI) induced and complicated by leukostasis. Immunophenotyping, morphology, cytogenetic and fluorescence-in-situ-hybridization analysis revealed the diagnosis of a blastoid variant of MCL. To remove leukocytes rapidly, leukapheresis was performed in the intensive care unit. Based on the differential blood count with 95% blasts, which were assigned to the lymphocyte population by the automatic hematology analyzer, leukapheresis procedures were then performed with the mononuclear cell standard program on the Spectra cell separator. The patient was treated with daily leukapheresis for 3 days. The WBC count decreased to 174 × 10(9)/L after the third leukapheresis, with a 72% reduction. After the second apheresis, treatment with vincristine, cyclophosphamide, and prednisolone was started. The patient fully recovered in the further course of the treatment. To the best of our knowledge, this is the first report on blastoid MCL with leukostasis associated with a STEMI that was successfully treated by leukapheresis. Effective harvest of circulating lymphoma cells by leukapheresis requires adaptation of instrument settings based on the results of the differential blood count prior to apheresis.

SUBMITTER: Nguyen XD

PROVIDER: S-EPMC3201628 | biostudies-other | 2011

REPOSITORIES: biostudies-other

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Project description:Acute abdominal syndrome can be caused by several possible reasons. The most common causes are perforation of a gastroduodenal ulcer, peritonitis, intestinal obstructions, and perforation of an appendix or fallopian tube. Fever and pain can be caused by an appendicitis or sigmoiditis. Appendiceal lymphoma is a rare disease that is usually found incidentally during appendectomy. Most of the cases are non-Hodgkin's lymphomas. Mantle cell lymphoma is an aggressive B-cell non-Hodgkin's lymphoma with a poorer prognosis than other B-cell lymphomas; thus, a definitive diagnosis is essential. A 60-year-old man presented with right lower quadrant pain. He denied any nausea, vomiting or anorexia and was afebrile. The physical examination revealed right lower quadrant abdomen tenderness. The computed tomography scan revealed periappendiceal fatty stranding with a swollen appendix, approximately 2 cm in diameter and prominent paraaortic, portacaval and mesenteric lymph nodes. A diagnosis of acute appendicitis was made, and laparoscopic appendectomy was performed immediately. The subsequent pathological examination revealed severe congestion with lymphoid hyperplasia. The immunohistochemistry stains revealed positive staining for cluster of differentiation (CD) CD20, B-cell lymphoma-2 (Bcl-2), cyclin D1, SRY-box transcription factor-11 (SOX-11), immunoglobulin D (IgD) and immunoglobulin M (IgM) but negative staining for CD3, CD5, CD10 and CD23. 18F-FDG positron emission tomography showed peripheral lymph node involvement, while the bone marrow biopsy showed negative findings. Therefore, a diagnosis of mantle cell lymphoma, Ann Arbor stage IVA, was made. The patient received postoperative combination chemotherapy and remained in a stable condition over a 1-year follow-up period. We report an uncommon case that initially presented as acute appendicitis, for which a final diagnosis of mantle cell lymphoma was made. In comparison with other B-cell lymphomas, mantle cell lymphoma has a poorer prognosis, and positive immunochemical staining of cyclin D1 and SOX-11 is useful for differentiating mantle cell lymphoma from other appendiceal lymphomas and treating patients appropriately. Physicians and nursing staff should be also aware of the associated complications and management in these patients.

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Rapid treatment of leukostasis in leukemic mantle cell lymphoma using therapeutic leukapheresis: a case report.

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