Clonality of Acquired Primary Pure Red Cell Aplasia: Effectiveness of Antithymocyte Globulin

Dınçol, Günçağ; Aktan, Melih; Nalçacı, Meliha; Yavuz, Akif Selim; Keskin, Hüseyin Levent; Dawson, Brian; Dinçol, Koray

doi:10.3109/10428190109097772

Cited by 7 publications

(3 citation statements)

References 14 publications

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“…7 Primary PRCA outside the context of marrow transplantation has been attributed to humoral dysfunction producing autoantibodies against erythroid cells, or T cell-mediated inhibition of erythropoiesis. 8 Clonal TCR rearrangements have been reported in idiopathic PRCA 9 and were detected in the peripheral blood in our patient prior to the onset of PRCA and 8 weeks thereafter, but subsequently resolved. While oligoclonal expression of T cells after allografting is well recognised, with persistence particularly of CD8 þ T cell clones, 10 many T cells post transplant have abnormal phenotypes.…”

Section: Discussionsupporting

confidence: 49%

Pure red cell aplasia with the onset of graft versus host disease

Grigg

Juneja

2003

Bone Marrow Transplant

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Summary:Pure red cell aplasia (PRCA) occurred in the fourth month after an ABO-compatible nonmyeloablative allograft coincident with the cessation of immunosuppression and the onset of limited chronic GVHD. No secondary causes could be identified. Erythropoiesis was restored promptly and durably with the resumption of immunosuppression. A clonal T cell receptor c rearrangement was detected in peripheral blood lymphocytes prior to the onset of PRCA. PRCA should be added to the list of immunohaematological complications of GVHD. Bone Marrow Transplantation (2003) 32, 1099-1101. doi:10.1038/sj.bmt.1704271 Keywords: pure red cell aplasia; GVHD Pure red cell aplasia (PRCA) is most commonly an idiopathic disorder, although secondary causes such as lymphoproliferative and myelodysplastic disorders, parvovirus infection and thymoma are well recognised. 1 Post transplant, PRCA is usually a complication of major ABO incompatibility and may have an increased incidence after nonmyeloablative compared with myeloablative conditioning regimens. 2 While autoimmune haematological disorders including haemolytic anaemia and immune neutropenia, thrombocytopenia and pancytopenia due to peripheral destruction are well recognised post allograft, 3 often in the context of chronic graft versus host disease (GVHD), 4 to our knowledge PRCA as an immune complication in the absence of ABO incompatibility has not been reported. This case documents PRCA after an ABO-compatible nonmyeloablative allograft in the absence of any secondary causes. The onset was coincident with the early onset of chronic GVHD and it resolved promptly after the recommencement of immunosuppression. Case reportA 48-year-old man underwent a peripheral blood progenitor cell, ABO-matched sibling allograft in May 2002 for transformed low-grade non-Hodgkin's lymphoma relapsing after an autograft 11 months previously. A reducedintensity conditioning regimen (fludarabine 25 mg/m 2 day 6 to day 2, cyclophosphamide 1 g/m 2 day 3 to day 2) was used with cyclosporin A 3 mg/kg i.v. and short course methotrexate as GVHD prophylaxis. White cell recovery occurred early in the second week post transplant (neutrophils 40.5 Â 10 9 /l by day 10). Mild early cutaneous acute GVHD was observed requiring low-dose steroid therapy. Both cyclosporin A and prednisolone were tapered from day 60 and stopped by day 100. Table 1 details the haemoglobin (Hb) levels, marrow findings, chimerism results, immunosuppressive medication and GVHD status at various time points post transplant. Hb levels were stable around 120 g/l without transfusion by day 95, but fell thereafter by approximately 1 g/week to a nadir of 78 g/l by day 120. There was no history of blood loss. Red cell aplasia, confirmed by glycophorin immunohistochemistry, was demonstrated on a day 100 marrow aspirate ( Figure 1a). No B cell lymphoma was demonstrated by morphology or flow cytometry. The majority of marrow lymphocytes were suppressor T cells with a CD4:CD8 ratio of 0.5. An excess of large granular lymphocytes was not seen in the...

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Section: Discussionsupporting

confidence: 49%

Pure red cell aplasia with the onset of graft versus host disease

Grigg

Juneja

2003

Bone Marrow Transplant

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“…A clear correlation exists between the development of auto‐immune phenomena such as hypogammaglobulinemia, MG, AA, and PRCA, and neoplasms of the cortical thymus (1). This association arises from the capacity for thymomas to generate mature CD4 + and CD8 + T cells from immature precursors, and in doing so produce the auto‐reactive T cell clones responsible for humoral and cytotoxic auto‐immune disease (8, 9).…”

Section: Discussionmentioning

confidence: 99%

Aplastic anemia as a late complication of thymoma in remission

Ritchie

Underhill²,

Grigg

2002

European J of Haematology

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Thymoma has many auto-immune associations, including aplastic anemia and myasthenia gravis. The pathophysiologic relationship between thymoma and its auto-immune sequelae are yet to be fully elucidated, but are thought to be as a result of the production of auto-reactive T cell clones by the thymic epithelium. We report a case in which aplastic anemia did not develop until sometime after remission from thymoma had been induced. This observation suggests that auto-reactive T cells may be produced by the thymoma but not induce clinical auto-immune disease until a later time, even after eradication of thymoma has been achieved.

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“…Table 3 . shows the available evidence for efficacy of ATG in PRCA [ 19 , 20 , 21 , 22 , 23 , 24 , 25 ].…”

Section: Atg For Acquired Pure Red Cell Aplasiamentioning

confidence: 99%

Anti Thymocyte Globulin-Based Treatment for Acquired Bone Marrow Failure in Adults

Tjon

Langemeijer

Halkes

2021

Cells

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Idiopathic acquired aplastic anemia can be successfully treated with Anti Thymocyte Globulin (ATG)-based immune suppressive therapy and is therefore considered a T cell-mediated auto immune disease. Based on this finding, several other forms of idiopathic acquired bone marrow failure are treated with ATG as well. For this review, we extensively searched the present literature for evidence that ATG can lead to enduring remissions in different forms of acquired multi- or single-lineage bone marrow failure. We conclude that ATG-based therapy can lead to an enduring hematopoietic response and increased overall survival (OS) in patients with acquired aplastic aplasia. In patients with hypocellular myelodysplastic syndrome, ATG can lead to a hematological improvement without changing the OS. ATG seems less effective in acquired single-lineage failure diseases like Pure Red Cell Aplasia, Amegakaryocytic Thrombocytopenia and Pure White Cell Aplasia, suggesting a different pathogenesis in these bone marrow failure states compared to aplastic anemia. T cell depletion is hypothesized to play an important role in the beneficial effect of ATG but, as ATG is a mixture of polyclonal antibodies binding to different antigens, other anti-inflammatory or immunomodulatory effects could play a role as well.

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Clonality of Acquired Primary Pure Red Cell Aplasia: Effectiveness of Antithymocyte Globulin

Cited by 7 publications

References 14 publications

Pure red cell aplasia with the onset of graft versus host disease

Pure red cell aplasia with the onset of graft versus host disease

Aplastic anemia as a late complication of thymoma in remission

Anti Thymocyte Globulin-Based Treatment for Acquired Bone Marrow Failure in Adults

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