Literature DB >> 2914601

A myelokathexis-like variant of myelodysplasia.

S M Rassam1, P Roderick, I al-Hakim, A V Hoffbrand.   

Abstract

A 34-year-old woman presented with pancytopenia, profound neutropenia and repeated infections. A bone marrow specimen revealed hypercellularity with trilineage dysplasia and bizarre granulocytic morphology identical to that of the rarely reported myelokathexis. Investigations revealed defects in platelet and neutrophil functions and markedly reduced colony-to-cluster ratio in bone marrow culture. She required treatment with antibiotics, surgical intervention for a gangrenous abdominal wall, and eventually underwent a successful allogeneic bone marrow transplant from her sister. This case suggests that myelokathexis is an unusual form of myelodysplasia with a relatively benign course and presenting at a young age.

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Year:  1989        PMID: 2914601     DOI: 10.1111/j.1600-0609.1989.tb00255.x

Source DB:  PubMed          Journal:  Eur J Haematol        ISSN: 0902-4441            Impact factor:   2.997


  4 in total

Review 1.  Genetics on a WHIM.

Authors:  Omar Al Ustwani; Razelle Kurzrock; Meir Wetzler
Journal:  Br J Haematol       Date:  2013-09-20       Impact factor: 6.998

Review 2.  WHIM Syndrome: from Pathogenesis Towards Personalized Medicine and Cure.

Authors:  Lauren E Heusinkveld; Shamik Majumdar; Ji-Liang Gao; David H McDermott; Philip M Murphy
Journal:  J Clin Immunol       Date:  2019-07-16       Impact factor: 8.317

3.  Bone microstructure is significantly altered in CRPS-affected distal tibiae as detected by HR-pQCT: a retrospective cross-sectional study.

Authors:  Nicola Oehler; Tim Rolvien; Tobias Schmidt; Sebastian Butscheidt; Ralf Oheim; Florian Barvencik; Haider Mussawy
Journal:  J Bone Miner Metab       Date:  2018-11-21       Impact factor: 2.626

4.  Familial occurrence of warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome.

Authors:  Maciej Siedlar; Zbigniew Rudzki; Magdalena Strach; Elzbieta Trzyna; Anna Pituch-Noworolska; Anita Błaut-Szlósarczyk; Karolina Bukowska-Strakova; Marzena Lenart; Tomasz Grodzicki; Marek Zembala
Journal:  Arch Immunol Ther Exp (Warsz)       Date:  2008-12-01       Impact factor: 4.291

  4 in total

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