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01-08-2009 | Short Report

Transient myelofibrosis with autoimmune pancytopenia: a case report

Authors: Tomohei Nakao, Takashi Fukushima, Takashi Shimizu, Toru Nanmoku, Satoshi Fujiyama, Ryoko Nakajima, Fujiko Fukushima, Masayuki Noguchi, Ryo Sumazaki

Published in: European Journal of Pediatrics | Issue 8/2009

Abstract

Introduction

Myelofibrosis associated with myelodysplasia is thought to herald poor prognosis in myelodysplastic syndrome (MDS).

Case report

A 7-month-old boy presented with fever (39°C), pancytopenia, and slight hepatosplenomegaly (3 and 2 cm, respectively). Bone marrow showed hypercellularity, hyperplasia of erythroblasts, and also myelofibrosis. IgG was 1,136 mg/dL, IgA was 131 mg/dL, and IgM was 89 mg/dL. Antinuclear and antineutrophil antibodies, red-blood-cell-associated IgG, antiplatelet antibodies, and Coombs test were positive. Karyotype was 46XY. No viral cause was evidenced. Mild myelodysplasia was revealed two months later, but was insufficient to support a diagnosis of MDS. The boy was treated with transfusion of packed cells, prednisolone 2 mg/kg/day for 3 weeks associated with intravenous gammaglobulin 400 mg/kg/day for 5 days. Direct Coombs remained positive 1 month after treatment for 5 months, myelofibrosis persisted for 3 months, and neutropenia for 21 months. After 3-year follow-up, hematological data were normal without any therapeutic intervention.

Conclusion

Myelofibrosis associated with mild myelodysplasia and pancytopenia can have a benign evolution in infants and young children.

Altura RA, Head DR, Wang WC (2000) Long-term survival of infants with idiopathic myelofibrosis. Br J Haematol 109:459–462PubMedCrossRef

Barcellini W, Zaninoni A, Imperiali FG et al (2007) Anti-erythroblast autoimmunity in early myelodysplastic syndromes. Haematologica 92:19–26PubMedCrossRef

Bourgeosis E, Caulier MT, Rose C et al (2001) Role of splenectomy in the treatment of myelodysplastic syndromes with peripheral thrombocytopenia: a report on six cases. Leukemia 15:950–953CrossRef

Boxer LA, Camitta BM, Berenberg W et al (1975) Myelofibrosis-myeloid metaplasia in childhood. Pediatrics 55:861–865PubMed

Carroll WL, Berberich FR, Glader BE (1986) Pancytopenia with myelofibrosis. An unusual presentation of childhood Hodgkin’s disease. Clin Pediatr (Phila) 25:106–108

Chabannon C, Molina L, Peqourie-Bandelier B et al (1994) A review of 76 patients with myelodysplastic syndromes treated with danazol. Cancer 73:3073–3080PubMedCrossRef

George JN, Woolf SH, Raskob GE et al (1996) Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood 88:3–40PubMed

Hebbar M, Kaplan C, Caulier MT et al (1996) Low incidence of specific anti-platelet antibodies detected by the MAIPA assay in the serum of thrombocytopenic MDS patients and lack of correlation between platelet autoantibodies, platelet lifespan and response to danazol therapy. Br J Haematol 94:112–115PubMedCrossRef

Jeje MO, Blajchman MA, Steeves K et al (1984) Quantitation of RBC-associated IgG using immunoradiometric assay. Transfusion 24:473–476PubMedCrossRef

10.

Labotka RJ, Morgan ER (1982) Myelofibrosis in neuroblastoma (1982). Med Pediatr Oncol 10:21–26PubMedCrossRef

11.

Lin JT, Wang WS, Yen CC et al (2002) Myelodysplastic syndrome complicated by autoimmune hemolytic anemia: remission of refractory anemia following mycophenolate mofetil. Ann Hematol 81:723–726PubMedCrossRef

12.

Luiken GA, McMillan R, Lightsey AL et al (1977) Platelet-associated IgG in immune thrombocytopenic purpura. Blood 50:317–325PubMed

13.

Luna-Fineman S, Shannon KM, Atwater SK et al (1999) Myelodysplastic and myeloproliferative disorders of childhood: a study of 167 patients. Blood 93:459–466PubMed

14.

Marisavljevic D, Rolovic Z, Cemerikic V et al (2004) Myelofibrosis in primary myelodysplastic syndromes. Med Oncol 21:325–331PubMedCrossRef

15.

Maschek H, Georgii A, Kalotsi V et al (1992) Myelofibrosis in primary myelodysplastic syndromes: a retrospective study of 352 patients. Eur J Haematol 48:208–214PubMedCrossRef

16.

McCarthy DM (1985) Fibrosis of the bone marrow: content and causes. Br J Haematol 59:1–7PubMedCrossRef

17.

Meager A, Visvalingam K, Peterson P et al (2006) Anti-interferon autoantibodies in autoimmune polyendocrinopathy syndrome type 1. PLOS Med 3:1152–1164CrossRef

18.

Michel G, Thuret I, Capodano AM et al (1991) Myelofibrosis in a child suffering from a hypereosinophilic syndrome with trisomy 8: response to corticotherapy. Med Pediatr Oncol 19:62–65PubMedCrossRef

19.

Naithani R, Tyagi S, Choudhry VP (2008) Secondary myelofibrosis in children. J Pediatr Hematol Oncol 30:196–198PubMedCrossRef

20.

Nakahata T, Kojima S, Tsuchida M et al (1999) The MDS Committee of The Japanese Society of Pediatric Hematology. A guide to diagnosis of childhood myelodysplastic syndromes. Japanese Journal of Pediatric Hematology [Japanese] 13:381–393

21.

Noren-Nystrome U, Roos G, Bergh A et al (2008) Bone marrow fibrosis in childhood acute lymphoblastic leukemia correlates to biological factors, treatment response and outcome. Leukemia 22:504–510CrossRef

22.

Oren H, Ucar C, Gullen H et al (2001) Autoimmune hemolytic anemia occurring with myelodysplastic syndrome: report of a pediatric case and review of the literature. Ann Hematol 80:540–542PubMedCrossRef

23.

Pilorget H, Banqui A, Adam M, Leverger G (1996) Myelofibrosis regressing under corticotherapy and intravenous immunoglobulins in an infant. Arch Pediatr 3:40–43PubMedCrossRef

24.

Schwartz RS (1995) Autoimmune hemolytic anemias. In: Hoffman R, Benz EJ, Shattil SJ, Furie B, Cohen HJ (eds) Hematology: basic principles and practice, 2nd edn. Churchill Livingstone, New York, NY, pp 710–729

25.

Sokol RJ, Hewitt S, Booker DJ (1989) Erythrocyte autoantibodies, autoimmune haemolysis, and myelodysplastic syndromes. J Clin Pathol 42:1088–1091PubMedCrossRef

26.

Tamaki H, Ogawa H, Ohyashiki K et al (1999) The Wilms’ tumour gene, WT1 is a good marker for diagnosis of disease progression of myelodysplastic syndromes. Leukemia 13:393–399PubMedCrossRef

27.

van der Vliet HJJ, Nieuwenhuis EE (2007) IPEX as a result of mutations in FOXP3. Clin Dev Immunol 89017

28.

Ware R, Kinney TR, Friedman HS et al (1986) Prognostic implications for direct platelet-associated IgG in childhood idiopathic thrombocytopenic purpura. Am J Pediatr Hematol Oncol 8:32–37PubMedCrossRef

Title: Transient myelofibrosis with autoimmune pancytopenia: a case report
Authors: Tomohei Nakao
Takashi Fukushima
Takashi Shimizu
Toru Nanmoku
Satoshi Fujiyama
Ryoko Nakajima
Fujiko Fukushima
Masayuki Noguchi
Ryo Sumazaki
Publication date: 01-08-2009
Publisher: Springer-Verlag
Published in: European Journal of Pediatrics / Issue 8/2009
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-008-0867-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Transient myelofibrosis with autoimmune pancytopenia: a case report

Abstract

Introduction

Case report

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Case report

Conclusion

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