Pure Red-Cell Aplasia in Patients with Chronic Lymphocytic Leukemia

Chikkappa, G.; Zarrabi, Mahboobeh; Tsan, Min‐Fu

doi:10.1097/00005792-198609000-00006

Cited by 66 publications

(35 citation statements)

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“…In a large study [4] of 2333 consecutive patients with myelofi brosis with myeloid metaplasia (MMM), 91 were reported to undergo leukemic transformation (LT) of myeloid origin (acute myeloid leukemia, AML) with all the subtypes except AML, M3. Although the association of PRCA with chronic lymphocytic leukemia [5] and large granular lymphocytic leukemia [6] has been described, however its progression to acute leukemia is rare. There is only one case report [7] describing the progression of PRCA through hyperfi brotic MDS to acute myeloid leukemia.…”

Section: Discussionmentioning

confidence: 99%

PRCA with myelofibrosis: an unusual case report

Kar

Mahapatra

Pati

2008

Indian J Hematol Blood Transfus

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Section: Discussionmentioning

confidence: 99%

PRCA with myelofibrosis: an unusual case report

Kar

Mahapatra

Pati

2008

Indian J Hematol Blood Transfus

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“…Primary pure red cell aplasia is treated as an immunologically mediated disease, based on a number of studies implicating a pathogenic role for serum autoantibodies or T lymphocytes (Dessypris, 1991). In the case of pure red cell aplasia secondary to chronic lymphocytic leukaemia of the B-or T-cell type, immunologic mechanisms also have been implicated in the pathogenesis of the erythroid aplasia (Chikkappa et al, 1986;Nagasawa et al, 1981).…”

Section: Discussionmentioning

confidence: 99%

Cyclophosphamide therapy for pure red cell aplasia associated with granular lymphocyte‐proliferative disorders

1997

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Granular lymphocytes have been characterized as cells with azurophilic granules in the cytoplasm. Patients with increased numbers of granular lymphocytes are designated as granular lymphocyte‐proliferative disorders (GLPDs). A variety of haematological abnormalities are associated with T‐cell‐lineage GLPD. Among these, pure red cell aplasia is frequent, and adequate therapy is required. Seven patients with pure red cell aplasia, or a related condition complicating T‐cell‐lineage GLPD, were entered into this study. Cyclophosphamide was initiated at a daily oral dose of 100 mg. After 2 weeks the dose was reduced to 50 mg/d, and maintained at that dose. Cyclophosphamide was administered until the lymphocyte count was <1 ×109 l and T‐cell receptor‐β gene analysis was used to monitor the response to treatment. All the patients were successfully treated, irrespective of their former treatment. Clinical remission was associated with the disappearance of the abnormal granular lymphocyte clone, as detected by Southern blot hybridization analysis. Therapeutic responses began after 8 weeks, and clinical complete remissions were obtained after 6 months. Oral cyclophosphamide monotherapy can successfully treat the pure red cell aplasia associated with T‐cell‐lineage GLPD.

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“…1 We report a patient who pressented with PRCA as the first manifestation of CLL, which was refractory to sequential treatments with steroids, i.v. immunoglobulins, cyclosporin A and alkylating agents and eventually responded to a combination of fludarabine followed by cyclosporin A and erythropoietin (Epo).…”

Section: To the Editormentioning

confidence: 99%

“…PRCA is a rare complication of CLL. 1 It is most commonly associated with B CLL but has also been reported in T cell CLL and other lymphoproliferative disorders. Suppressor/ cytotoxic T cells have been shown to exert a suppressive effect on erythropoiesis in CLL.…”

Section: Cd20mentioning

confidence: 99%