Splenic Lymphoma Presenting as Warm Autoimmune Hemolytic Anemia Associated with Pure Red Cell Aplasia

Zeidman, Aliza; Fradin, Zinaida; Barac, Y.; Bendayan, D.; Mittelman, Moshe; Orlin, Jerome

doi:10.1046/j.1423-0410.2000.7820126.x

Cited by 15 publications

(6 citation statements)

References 29 publications

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“…2,3,6,16 -18,21 The patients' ages were variable (median, 50 years; range, 20 -78 years). No significant difference in incidence by gender has been identified (12 males including our case 6 -8,13,15,17,19,20 and 11 females 3,4,6,11,16,18,21,22 ). Fifteen reported cases discuss the onset of NHL and PRCA: seven developed PRCA and NHL and cellular 4,33 inhibitors of erythropoiesis, and serum derived from lymphoma patients accompanied by PRCA has been shown to inhibit erythroid colony formation in vitro.…”

Section: Case Reportcontrasting

confidence: 48%

“…Fifteen reported cases discuss the onset of NHL and PRCA: seven developed PRCA and NHL simultaneously. 6,8,9,18,19,21 In three cases, PRCA preceded NHL; the intervals from the onset of PRCA to that of NHL were 4, 4 43 16 and 180 22 months. Five cases developed PRCA after the onset of NHL, at intervals of 2, 3 5, 2 5 7 and 15 15 months; in one case the interval was not reported.…”

Section: Discussionmentioning

confidence: 92%

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Pure Red Cell Aplasia and Myelofibrosis in B-cell Neoplasm

et al. 2005

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We describe an unusual case of B-cell neoplasm accompanied by pure red cell aplasia (PRCA) and myelofibrosis in a 67-year-old male presenting with severe anaemia. A few unclassified, myeloperoxidase-negative blastoid cells were seen on bone marrow aspiration, and erythroid cell hypoplasia and myelofibrosis on bone marrow biopsy. An autoimmune PRCA was suspected, as serum CH50, C3 and C4 levels were consistently low. Ciclosporin was effective in treating the anaemia, but anaemia returned when the drug was discontinued.Thirteen months later, the patient was admitted with pleural effusion and ascites that contained monoclonal CD19+ CD20+ immature blast cells with a complex karyotype, thought to be neoplastic B-cells. The unclassified blastoid cells seen earlier may therefore have been from the same origin. The patient deteriorated rapidly and died. Only one case of non-Hodgkin's lymphoma with PRCA and myelofibrosis has been reported previously. We discuss the possibility that dysregulated T-cells induced by neoplastic B-cells may have given rise to concomitant PRCA and myelofibrosis.

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Section: Case Reportcontrasting

confidence: 48%

Section: Discussionmentioning

confidence: 92%

Pure Red Cell Aplasia and Myelofibrosis in B-cell Neoplasm

et al. 2005

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“…There are several reports of cases in which both AIHA and PRCA occurred simultaneously or sequentially (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19). Table is a list of the published reports.…”

Section: Discussionmentioning

confidence: 99%

“…Table is a list of the published reports. Usually, this occurs in association with other diseases, including: hematological malignancies such as lymphoma (6,8,20) and chronic lymphocytic leukemia (15); autoimmune diseases such as Sjögren syndrome (9,11,17) and SLE (13); infections such as hepatitis (19); or due to the use of immunosuppressive drugs after transplantation, such as alemtuzumab (10,14). Although rare, cases of primary AIHA followed by PRCA that are not associated with other have been reported (3,7,12,16).…”

Section: Discussionmentioning

confidence: 99%

Coombs-negative Autoimmune Hemolytic Anemia Followed by Anti-erythropoetin Receptor Antibody-associated Pure Red Cell Aplasia: A Case Report and Review of Literature

et al. 2016

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A 76-year-old woman was referred to our hospital because of anemia. The laboratory findings revealed hemolysis. Although a direct Coombs test was negative, a high titer of RBC-bound IgG was detected, and a diagnosis of Coombs-negative autoimmune hemolytic anemia was made. She was successfully treated with prednisolone. One year and five months later, she again presented anemia and was diagnosed with pure red cell aplasia. Anti-erythropoietin receptor antibody was detected in the serum. She was treated with cyclosporine and obtained prompt recovery. We herein report this rare case and review the pertinent literature.

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“…In one-third of cases a monoclonal IgM paraprotein is detectable and autoimmune phenomena like autoimmune hemolytic anemia, lupus anticoagulant and other acquired inhibitors to clotting factors have been described [6]. PRCA secondary to SMZL has been reported in the literature in only two cases so far [7,8], only one of whom was treated with rituximab [7]. SMZL is treated by splenectomy, a procedure aiding not only in debulking disease burden but also in reducing marrow infiltration.…”

Section: Discussionmentioning

confidence: 99%

Pure red cell aplasia as first manifestation of splenic marginal zone lymphoma-successful treatment with rituximab: a case report

Anastasiadis

Margaritis

Kotsianidis

et al. 2009

Cases J

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IntroductionAcquired pure red cell aplasia is a rare disorder, usually appearing secondary to various pathologic conditions such as thymoma, systemic autoimmune diseases or in the course of lymphomas. Conventional treatment consists of immunosuppression with corticosteroids, antithymocyte globulin or cyclosporin-A.Case presentation8 weekly courses of rituximab were administered to a patient who presented with pure red cell aplasia secondary to newly diagnosed splenic marginal zone lymphoma. Transfusion independence was achieved after the 6th course, and pure red cell aplasia receded completely with therapy.ConclusionPure red cell aplasia may ensue early in the course of splenic marginal zone lymphoma and other low grade lymphomas. Rituximab is a safe and effective alternative treatment for pure red cell aplasia secondary to lymphoproliferative disorders.

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Splenic Lymphoma Presenting as Warm Autoimmune Hemolytic Anemia Associated with Pure Red Cell Aplasia

Cited by 15 publications

References 29 publications

Pure Red Cell Aplasia and Myelofibrosis in B-cell Neoplasm

Pure Red Cell Aplasia and Myelofibrosis in B-cell Neoplasm

Coombs-negative Autoimmune Hemolytic Anemia Followed by Anti-erythropoetin Receptor Antibody-associated Pure Red Cell Aplasia: A Case Report and Review of Literature

Pure red cell aplasia as first manifestation of splenic marginal zone lymphoma-successful treatment with rituximab: a case report

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