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Annals of Hematology

, Volume 87, Issue 12, pp 1019–1020 | Cite as

Primary myelofibrosis in a patient who developed primary biliary cirrhosis, autoimmune hemolytic anemia and fibrillary glomerulonephritis

  • L. I. Kornblihtt
  • P. S. Vassalllu
  • P. G. Heller
  • N. R. Lago
  • C. L. Alvarez
  • F. C. Molinas
Letter to the Editor

Dear Editor,

We report a patient with primary myelofibrosis (PMF) who developed primary biliary cirrhosis, autoimmune hemolytic anemia, and fibrillary glomerulonephritis (FGN).

A 67-year-old woman was referred to our institution in November 1999 because of asthenia and nocturnal sweats associated to PMF, which was diagnosed in September 1996. In June 1998, high liver enzymes, circulating antimitochondrial antibodies, and a liver biopsy disclosed primary biliary cirrhosis. She had a history of arthralgia, positive latex test, xerosthalmia, and xerosthomia.

On admission, she presented with hepatosplenomegaly, moderate thrombocytosis, mild anemia, elevated leukocytes and 6% circulating blasts. Leukocyte alkaline phosphatase score was very low, karyotype was normal, no bcr-abl translocation was detected, and high lactic dehydrogenase levels were found. Bone marrow (BM) aspiration yielded a dry tap and BM biopsy disclosed megakaryocyte and myeloid hyperplasia, diffuse increase in reticulin...

Keywords

Primary Biliary Cirrhosis Autoimmune Hemolytic Anemia JAK2V617F Mutation Osteosclerosis Primary Myelofibrosis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

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    Paquette RL, Meshkinpour A, Rosen PJ (1994) Autoimmune myelofibrosis. A steroid-responsive cause of bone marrow fibrosis associated with systemic lupus erythematosus. Medicine (Baltimore) 73:145–152. doi: 10.1097/00005792-199405000-00003 Google Scholar
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    Rosenstock J, Markowitz GS, Valeri AM et al (2003) Fibrillary and immunotactoid glomerulonephritis: distinct entities with different clinical and pathologic features. Kidney Int 63:1450. doi: 10.1046/j.1523-1755.2003.00853.x PubMedCrossRefGoogle Scholar
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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • L. I. Kornblihtt
    • 1
  • P. S. Vassalllu
    • 2
  • P. G. Heller
    • 1
  • N. R. Lago
    • 4
  • C. L. Alvarez
    • 3
  • F. C. Molinas
    • 1
  1. 1.Seccción Hematología InvestigaciónInstituto de Investigaciones Médicas Alfredo LanariBuenos AiresArgentina
  2. 2.Seccción Hematología, Instituto de Investigaciones Médicas Alfredo Lanari, Facultad de MedicinaUniversidad de Buenos AiresBuenos AiresArgentina
  3. 3.Sección Patología, Instituto de Investigaciones Médicas Alfredo Lanari, Facultad de Medicina Universidad de Buenos AiresBuenos AiresArgentina
  4. 4.Centro de Patología Experimental, Departamento de Patología, Facultad de MedicinaUniversidad de Buenos AiresBuenos AiresArgentina

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