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Case Report Recovery of Erythropoiesis Following Allogeneic Bone Marrow Transplantation for Chronic Lymphocytic Leukaemia-Associated Pure Red Cell Aplasia

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Case Report Recovery of Erythropoiesis Following Allogeneic Bone Marrow Transplantation for Chronic Lymphocytic Leukaemia-Associated Pure Red Cell Aplasia Bone Marrow Transplantation (2001) 27, 771–773 2001 Nature Publishing Group All rights reserved 0268–3369/01 $15.00 www.nature.com/bmt Case report Recovery of erythropoiesis following allogeneic bone marrow transplantation for chronic lymphocytic leukaemia-associated pure red cell aplasia MP de Vetten, M van Gelder and GE de Greef Department of Hematology, University Hospital Rotterdam/Daniel den Hoed Cancer Center, Rotterdam, Netherlands Summary: toms associated with anaemia. Initial work up revealed: haemoglobin 4 mmol/l (6.4 g/dl), MCV 98 fl and reticulo- Pure red cell aplasia is a rare condition, that can be cytes 2 ϫ 109/l. The remainder of the haematology and either idiopathic or associated with a lymphoprolifer- biochemical laboratory studies were normal, with WBC of ative disorder. The latter is considered to result from T 9.3 ϫ 109/l and absolute lymphocyte count of 4.7 ϫ 109/l, cell-mediated suppression of haematopoiesis, and usu- as were levels of vitamin B12 and folic acid. There was ally responds well to treatment with immunosuppressive no serologic evidence of recent CMV or EBV infection, medication. We describe a patient with B-CLL-associa- serologic and DNA studies for parvovirus B19 infection ted pure red cell aplasia who did not respond to several were negative and diagnostic imaging studies showed no courses of immunosuppressive treatment. Erythro- evidence of thymoma. Bone marrow examination was poiesis was finally restored after allogeneic bone mar- remarkable for the near absence of mature erythroid precur- row transplantation. Bone Marrow Transplantation sors with paratrabecular clusters of mature lymphocytes on (2001) 27, 771–773. biopsy. Immunophenotyping showed a monoclonal B cell Keywords: pure red cell aplasia; allo-BMT; CLL population in blood and bone marrow (CD5ϩ, CD10Ϫ, CD19ϩ, CD20ϩ, CD23ϩ, weak expression of surface Ig), comprising approximately 35% of the lymphocyte popu- lation. Southern blot analysis of IgH genes confirmed the First described by Kazsnelson, pure red cell aplasia (PRCA) presence of a monoclonal B cell population, whereas no is a rare haematopoietic disorder characterised by selective clonal T cell receptor gene rearrangement was detected. 1,2 aplasia of the erythroid cell line in the bone marrow. The Cytogenetic analysis revealed a normal XY karyotype. In syndrome can be either congenital (Diamond–Blackfan vitro colony forming assays showed normal BFU-E and anaemia) or acquired. Most cases of acquired PRCA are CFU-GM from bone marrow nucleated cells as well as idiopathic, but a large number of associated illnesses have from T cell-depleted bone marrow mononuclear cells. Per- been described, most notably thymomas and lymphoid ipheral blood erythropoietin levels were elevated, but no 3 malignancies. The association of PRCA with B cell formal studies were done to exclude antibodies to erythro- chronic lymphocytic leukaemia (CLL) is well known, and poietin or erythropoietin receptors. A diagnosis of B-CLL, ␥ appears to be based on the presence of T suppressor RAI stage 0 with accompanying PRCA was made. 4,5 cells. Several studies report good results using immuno- Initial treatment with chlorambucil and prednisone fol- suppressive medication, in particular cyclosporin A, for the lowed by treatment with cyclosporin A and prednisone had 6,7 treatment of CLL-associated PRCA. We describe a no effect. Evaluation 1 year after diagnosis showed persist- patient with B-CLL-associated PRCA, who did not respond ent B-CLL (approximately 30% monoclonal B cells in bone to several courses of immunosuppressive treatment. marrow) and an ongoing transfusion requirement for red Erythropoiesis was finally restored after T cell-depleted cells. Subsequent treatment with antithymocyte globulin allogeneic bone marrow transplantation. (horse ATG 15 mg/kg/day for 5 days), cyclosporin A and steroids again had no effect on the transfusion require- ments. Treatment with cyclophosphamide, vincristine and Case report prednisone (CVP) and subsequently with three courses of fludarabine (25 mg/m2/day for 5 days) resulted in near com- A 43-year-old Caucasian man was first diagnosed with plete resolution of monoclonal B cells from blood and bone PRCA in 1997, when he presented with signs and symp- marrow, but had no effect on the PRCA. A short trial of erythropoietin and cyclosporin A again failed to reduce the transfusion requirement. Because of the ongoing need for Correspondence: Dr GE de Greef, Department of Hematology, University Hospital Rotterdam/Daniel den Hoed Cancer Center, PO Box 5201, 3008 red cell transfusions associated with an increase of serum AE Rotterdam, Netherlands ferritin, iron chelation therapy with desferroxamine was Received 25 August 2000; accepted 12 January 2001 initiated in 1999. Recovery of erythropoiesis after BMT MP de Vetten et al 772 Since alternative treatment options were lacking, allog- respond well to immunosuppressive therapy, occasional eneic bone marrow transplantation was considered to treat patients will fail even the most rigorous immune modu- the B-CLL-associated PRCA. Informed consent was lation. Orchard et al14 describe a patient with myeloma- obtained and after conditioning with cyclophosphamide (60 associated PRCA, who remained red cell transfusion- mg/kg/day for 2 days) and TBI (10 Gy in two fractions dependent, even after autologous stem cell transplantation. over 2 days with partial lung and kidney shielding), the We present a patient with CLL-associated PRCA who patient underwent a T cell-depleted allogeneic bone marrow failed to respond to all standard treatment modalities transplant with marrow from his HLA-identical brother. including combined immunosuppressive therapy with ATG The final graft composition was 1.32 ϫ 106 CD34ϩ cells/kg and cyclosporin A. We were particularly concerned about and 2 ϫ 105 CD3ϩ cells/kg. Cyclosporin A was given to the onset of transfusion-related iron overload. Indeed, a prevent GVHD. liver biopsy performed right before transplantation con- Rapid engraftment ensued, with neutrophils Ͼ0.5 ϫ firmed the presence of early hepatic haemosiderosis. In 109/l, platelets Ͼ20 ϫ 109/l and no red cell transfusion view of his underlying malignancy, his young age and requirement by day ϩ25. Bone marrow examination around anticipating a gradual worsening of cardiac and hepatic that time showed good myelo- and megakaryopoiesis with reserve due to ongoing iron overload, we considered an persistent erythroid hypoplasia. Full donor haematopoiesis allogeneic BMT appropriate. The initial lack of erythroid was confirmed by PCR analysis of variable number of tan- engraftment could be due to recipient T cell-mediated rejec- dem repeat (VNTR) differences. He was readmitted on day tion (as is fairly common after T cell-depleted BMT for ϩ54 with nausea, diarrhoea, fever and recurrent anaemia aplastic anaemia). A possible association with the later with reticulocytopenia. An extensive search for an infec- diagnosed RSV infection cannot be excluded, as viral infec- tious aetiology yielded no cause and pathological examin- tions can interfere with engraftment, but this remains specu- ation of biopsies from the sigmoid and stomach showed no lative. Interestingly, good erythroid engraftment with docu- evidence of aGVHD. Symptoms gradually improved. On mentation of full donor haematopoiesis was achieved after day ϩ90 signs and symptoms of an upper respiratory tract recovery from the RSV infection and is presently ongoing. infection developed. Virologic examination of nasal wash- ings revealed respiratory syncytial virus (RSV) infection. Treatment with nebulized ribavirin was started, resulting in References complete resolution of symptoms. Interestingly, his anae- mia resolved during ribavirin treatment. Bone marrow 1 Kazsnelson P. Zur Entstehung er Bluttplatchen. Verh Dtsch examination on day ϩ100 showed 24% erythropoiesis. Ges Inn Med 1922; 34: 557–569. Evaluation 6 months post transplantation showed a stable 2 Dessypris EN. Pure red cell aplasia. In: Hoffman R, Benz EJ, haemoglobin (7.2 mmol/l; 8.3 g/dl) without any transfusion Shattil SJ et al (eds). Hematology Basic Principles and Prac- requirement and a normal reticulocyte count (40 ϫ 109/l). tice, 3rd edn. Churchill Livingstone: New York, 2000, PCR-VNTR analysis again confirmed 100% donor pp 342–354. chimaerism. 3 Young NS. Acquired pure red cell aplasia and amegakary- ocytic thrombocytopenia. In: Young NS, Alter BP (eds). Aplastic Anemia, Acquired and Inherited. WB Saunders: Philadelphia, 1994, pp 216–228. Discussion 4 Mangan KF, Chikkappa G, Farley PC. T gamma cells suppress growth of erythroid colony-forming units in vitro in the pure Autoimmune phenomena are known to occur frequently in red cell aplasia of B-cell chronic lymphocytic leukemia. J Clin B-CLL and are often directed against haematopoietic cells. Invest 1982; 70: 1148–1156. The available data suggest that T cell-mediated suppression 5 Chikkappa G, Zarrabi MH, Tsan M-F. Pure red-cell aplasia in of erythropoiesis represents a secondary event occurring patients with chronic lymphocytic leukemia. Medicine 1986; during the course of the disease.8 Normal growth of BFU- 65: 339–351. E in vitro (as was the case in our patient) also points 6 Chikkappa G, Pasquale D, Phillips PG et al. Cyclosporin A towards an immune-mediated inhibition of erythropoiesis, for the treatment of pure red cell aplasia in a patient with chronic lymphocytic leukemia. Am J Hematol 1987; 26: and is generally associated with a good response to 9 179–189. immunosuppressive therapy. 7 Finelli C, Bandini G, Ricci P et al. Cyclosporin A in idiopathic No curative treatment options are presently available for and CLL-associated pure red cell aplasia. Haematologica treating CLL, however, encouraging results have recently 1987; 72: 537–540. been reported on the use of allogeneic stem cell transplan- 8 Mangan KF, D’Alessandro L. Hypoplastic anemia in B cell tation for younger patients.10,11 Whereas bone marrow chronic lymphocytic leukemia: evolution of T cell-mediated transplantation has been successful in several cases of con- suppression of erythropoiesis in early-stage and late-stage dis- genital hypoplastic anaemia, only one case report describes ease.
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