2014, Number 2
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Rev Hematol Mex 2014; 15 (2)
Diminished Fibrosis of Bone Marrow with Thalidomide and Prednisone at Low Doses in Patients with Primary Myelofibrosis
Barrera-Chairez E, Ron-Magaña AL, Ron-Guerrero CS
Language: Spanish
References: 28
Page: 43-52
PDF size: 426.56 Kb.
ABSTRACT
Background: The primary myelofibrosis is a clonal myeloproliferative
and malignant disease; the diagnosis is made by excluding other diseases
leading to myelofibrosis. 50% of cases are associated with mutation of
the JAK2 gene. The only treatment that provides healing in some cases
is the transplantation of hematopoietic progenitor cells.
Objective: To determine the degree of decrease in marrow fibrosis with
the combination of low-dose of thalidomide and prednisone in patients
with primary myelofibrosis.
Patients and method: A clinical, open study included 11 patients with a
diagnosis of primary myelofibrosis from June 2009 to June 2011. Patients
were determined the degree of myelofibrosis by bone marrow biopsy
before and 6 months after treatment with thalidomide 50 mg daily in
combination with prednisone 5 mg every 12 hours continuously.
Results: Of the 11 patients 5 were female and 6 male, average age 63
years (43-83 years). Myelofibrosis grade II was observed in 1 patient
(9%), grade III in 9 patients (82%) and grade IV in 1 patient (9%).
JAK2 was determined in 9 patients and 5 of which were positive and
4 negative. Spleen size to diagnosis averaged 19 cm (14-24 cm). In all
patients to diagnosis it was seem a leukoerythroblastic frame, which
disappeared in 45% of patients (5/11) at 6 months of treatment. The risk
at study entry, according to the IWG for Myelofibrosis Research and
Treatment, was: high risk in 7 patients, intermediate 2 in three patients
and intermediate risk 1 in a patient. After treatment a decreased risk
was found in 82% of patients (9/11),
p ‹ 0.05.
Conclusions: In 73% of cases no change was observed in the degree of
myelofibrosis at 6 months of treatment; only in one patient (9%) response
was observed with reduced myelofibrosis grade II to grade I and another
patient showed progression of grade III to grade IV,
p not significant.
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