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 Blood, 1 May 2006, Vol. 107, No. 9, pp. 3486-3488.
Prepublished online as a Blood First Edition Paper on January 17, 2006; DOI 10.1182/blood-2005-08-3319.

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CLINICAL TRIALS AND OBSERVATIONS
Brief report

High levels of circulating CD34 cells, dacrocytes, clonal hematopoiesis, and JAK2 mutation differentiate myelofibrosis with myeloid metaplasia from secondary myelofibrosis associated with pulmonary hypertension

Uday Popat, Adaani Frost, Enli Liu, Yongli Guan, April Durette, Vishnu Reddy, and Josef T. Prchal

From the Divisions of Hematology/Oncology, Center for Cell and Gene Therapy, and Division of Pulmonary Medicine, Baylor College of Medicine, Houston, TX; Department of Blood and Marrow Transplantation, The University of Texas M. D. Anderson Cancer Center, Houston; Department of Pathology, University of Alabama, Birmingham; and Department of Pathophysiology, First School of Medicine Charles University, Prague, Czech Republic.

We studied 25 patients with myelofibrosis with myeloid metaplasia and 19 patients with secondary myelofibrosis associated with pulmonary hypertension (PH). In these 2 groups, we compared the peripheral-blood CD34 count, the clonality of granulocytes and platelets in peripheral blood, the mutational status of the JAK2 kinase gene, and the morphology of the peripheral blood and bone marrow. We found that the following were distinctive features of myelofibrosis with myeloid metaplasia but not of secondary myelofibrosis due to PH: high circulating CD34 cell count, the presence of clonal platelets and granulocytes and of peripheral-blood dacrocytes, and a JAK2 1849G>T (V617F) mutation. We conclude that these are intrinsic features of clonal progenitors present in patients with myelofibrosis due to myeloproliferative disorders and that these features are not due to the abnormal marrow architecture seen in secondary myelofibrosis.


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Related Article in Blood Online:

Is bone marrow fibrosis the real problem?
Ronald Hoffman and Mingjiang Xu
Blood 2006 107: 3421-3422. [Full Text] [PDF]

Related Letter in Blood Online:

Circulating CD34+ cells, pulmonary hypertension, and myelofibrosis
Gian Paolo Fadini, Marco Schiavon, Marcella Cantini, Angelo Avogaro, Carlo Agostini, Josef T. Prchal, and Uday Popat
Blood 2006 108: 1776-1777. [Full Text] [PDF]



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