Primary myelofibrosis

A rare myeloproliferative neoplasm characterized by stem-cell derived clonal over proliferation of mature myeloid lineages, such as erythrocytes, leukocytes, and megakaryocytes, with variable degrees of megakaryocyte atypia, associated with reticulin and/or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis, and abnormal cytokine expression.

Thrombocytopenia

A reduction in the number of circulating thrombocytes.


Total: 3

                      


(per page)
PMID (PMCID)
24307514
MALE Adult
Thalidomide treatment in a myelofibrosis patient with leukemia transformation.
Huang WH, Li MS, Chu SC, Wang TF, Kao RH, Wu YF.
Int J Hematol. 2014;99(2):188-92.
Thalidomide, an oral immunomodulatory drug, has been used effectively in the treatment of primary myelofibrosis, in which some patients could become transfusion independent, and showed improvement in thrombocytopenia and reduction in spleen size.
21617547
MALE Middle Aged
Reversal of transfusion dependence by tumor necrosis factor inhibitor treatment in a patient with concurrent rheumatoid arthritis and primary myelofibrosis.
Ong A, Quach H, Leech M.
J Clin Rheumatol. 2011;17(4):211-3.
We report a patient with seropositive rheumatoid arthritis with concurrent primary myelofibrosis, who had transfusion-dependent anemia and moderate thrombocytopenia that reversed during treatment with adalimumab.
12884816
MALE Middle Aged
[Fatal septic shock and rhabdomyolysis following transfusion of platelet concentrates contaminated with Streptococcus pneumoniae].
Katayama T, Kamiya M, Hoshina S, Masuoka H, Nishiwaki K, Sano K, Hagino T, Kobayashi M.
Rinsho Ketsueki. 2003;44(6):381-5.
We report on a 58-year-old male diagnosed as having primary myelofibrosis with thrombocytopenia, who died of fatal septic shock and rhabdomyolysis after platelet concentrates (PCs) transfusion.