T gamma (T gamma) cells suppress growth of erythroid colony-forming units in vitro in the pure red cell aplasia of B-cell chronic lymphocytic leukemia.

Mangan, Kenneth F.; Chikkappa, G.; Farley, Patrick C.

doi:10.1172/jci110713

Cited by 106 publications

(58 citation statements)

References 27 publications

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“…In addition, normal Ty cells have been shown to suppress in vitro erythroid (6,36) and granulocyte (56) colony expression. These observations are strengthened by clinical examples, such as the one presented in this report, where anemia or neutropenia is correlated with in vitro inhibition of erythroid (7)(8)(9)(10) or myeloid (11,13) differentiation by such suppressor cells.…”

Section: Discussionmentioning

confidence: 99%

Evidence for genetic restriction in the suppression of erythropoiesis by a unique subset of T lymphocytes in man.

et al. 1983

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A B S T R A C T The suppression of erythropoiesis by lymphocytes from patients with a T cell lymphoproliferative syndrome and pure erythrocyte aplasia has been previously demonstrated. To study the nature of the suppressor cell and possible genetic restriction of this suppression, we investigated a patient with severe anemia, splenomegaly, lymphocytosis, and erythroid aplasia. A 3-mo course of low-dose daily oral cyclophosphamide achieved a complete remission for over 12 mo. The surface phenotype of his lymphocytes was analyzed by means of antibodies to lineage, differentiation, and activation-specific surface antigens. The cells expressed mature T cell antigens T3, T8, and Tll, while lacking Ti. Immature T cell, B cell, and the monocyte-specific antigen Mo2 were absent, while Mol, a monocyte-associated antigen not normally expressed on T cells, was present. T1O and Ia expressed as activation antigens were also present. The cells, cryopreserved at diagnosis, were thawed and co-cultured in plasma clot with patient remission marrow samples at T cell/bone marrow ratios of 1:1 and 2:1.There was nearly 90% suppression of erythroid colonyforming unit expression and 60% suppression of erythroid burst-forming unit expression at 2:1 T cell to bone marrow ratios and somewhat less suppression at 1:1. Granulocyte/macrophage progenitor expression was unaffected. Erythroid progenitor differentiation This work was presented in part at the 24th Annual Meeting of the American Society of Hematology, Washington, DC, 4-7 December 1982. Dr. Nadler was supported by a grant from the Medical Foundation of Boston.Received for publication 13 December 1982 and in revised form 20 April 1983. in the marrows of two HLA identical siblings was similarly suppressed. The cells were co-cultured with the marrows of nine nonrelated donors to investigate the potential genetic restriction of this suppression. Colony suppression equal to that observed in the marrow of the patient and his siblings was found in studies of two partially HLA identical individuals. No suppression was detected in marrow co-cultures of two entirely HLA dissimilar individuals. These results show that suppression of erythropoiesis by a unique subset of T8, Mol, Ia-positive lymphocytes isolated from a patient with lymphocytosis and erythrocyte aplasia is genetically restricted.

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Section: Discussionmentioning

confidence: 99%

Evidence for genetic restriction in the suppression of erythropoiesis by a unique subset of T lymphocytes in man.

et al. 1983

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“…For the patients with PRCA in whom a humoral inhibitor cannot be demonstrated, direct suppression of erythropoiesis by clonal or non-clonal large granular lymphocytes (CD8 T cells or natural killers) is thought to be the major mechanism of pathogenesis [10]. However, in our case this mechanism seems unlikely.…”

Section: Discussionmentioning

confidence: 63%

“…The aplasia may precede or occur in the absence of other features of SLE disease activity, as in our patient. Various autoimmune mechanisms of PRCA have been described, such as autoantibodies directed against erythroid progenitors [8] or erythropoietin [9], or T or NK cells that inhibit erythropoiesis [10]. However, the presence of a serum inhibitor against erythroid progenitors, detected by cell culture studies, has only occasionally been reported in patients with SLE-associated PRCA [11].…”

Section: Discussionmentioning

confidence: 99%

Pure red cell aplasia in a Sjögren's syndrome/lupus erythematosus overlap patient

2003

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Hematological complications of systemic lupus erythematosus usually include anemia of chronic disease and peripheral destruction of blood cells. We describe the case of a young woman with Sjö gren's syndrome/lupus erythematosus overlap, mother of infant with congenital heart block, complicated by pure red cell aplasia. The patient was asymptomatic until the onset of severe anemia. A serum inhibitor of erythropoiesis was detected before the onset of immunosuppression. Bone marrow examination showed a low CD4:CD8 ratio, an immune defect possibly linked with the unrestrained production of antibodies against erythroid progenitor cells. Am. J. Hematol. 72:259-262, 2003.

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“…Of relevance, LGL disorders are most commonly found to be associated with PRCA (23)(24). Moreover, remission correlates with the clearance of these cells (14)(15)(16)(17). The case of an LGL leukemia-associated PRCA described in 1999 by Handgretinger et aI., further supports this data and provides a good model for CLL-associated PRCA pathogenesis (24).…”

Section: Pathogenesis Ofcllsassociated Prcamentioning

confidence: 69%

“…Many studies published from the late Seventies to the early Eighties reported experimental evidence of inhibition of eythropoiesis due to abnormal function of the so-called Ty cells with Fe receptor for IgG (CD16) in CLL-associated PRCA (14)(15)(16)(17)(18)(19)(20)(21)(22). These cells, subsequently renamed (LGL), large granular lymphocytes, were shown to be increased in percentage in the bone marrow of patients with B-CLL and PRCA and this was able to inhibit erythroid growth in vitro (14).…”

Section: Pathogenesis Ofcllsassociated Prcamentioning

confidence: 99%

Chronic Lymphocytic Leukemia-Associated Pure Red Cell Aplasia

D’Arena

Cascavilla

2009

Int J Immunopathol Pharmacol

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Pure red cell aplasia (PRCA) is a well-known marrow failure which may be acquired or constitutional/congenital, as the Diamond-Blackfan syndrome. Acquired PRCA may show as a primary hematological disorder or secondary to an associated disease, infection or drug. PRCA rarely complicates chronic lymphocytic leukemia (CLL), may occur anytime in the course of the disease and, in this context, it is a minority of total PRCA. Anemia due to PRCA in CLL patients must be carefully evaluated and differentiated from other causes (autoimmune hemolytic anemia, neoplastic lymphocyte infiltration of bone marrow, chemotherapy) that require a different therapeutic approach. PRCA is thought to be an immunologically mediated disorder, but there is no uniformity in the setting of the management. Immunosuppressive therapy is frequently given, such as steroids and cyclosporin-A. Recently, anecdotal cases have been published on the effectiveness of monoclonal antibodies rituximab and alemtuzumab.

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T gamma (T gamma) cells suppress growth of erythroid colony-forming units in vitro in the pure red cell aplasia of B-cell chronic lymphocytic leukemia.

Cited by 106 publications

References 27 publications

Evidence for genetic restriction in the suppression of erythropoiesis by a unique subset of T lymphocytes in man.

Evidence for genetic restriction in the suppression of erythropoiesis by a unique subset of T lymphocytes in man.

Pure red cell aplasia in a Sjögren's syndrome/lupus erythematosus overlap patient

Chronic Lymphocytic Leukemia-Associated Pure Red Cell Aplasia

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