De novo Leukemic Variant of Mast Cell Leukemia With KIT D816V

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De novo Leukemic Variant of Mast Cell Leukemia With KIT D816V

Mast cell leukemia (MCL), a highly aggressive form of systemic mastocytosis, accounts for less than 1% of all cases [1]. It is diagnosed on the basis of the criteria for systemic mastocytosis proposed by the WHO and the presence of at least 20% atypical mast cells of all bone marrow (BM) cells [2]. Here, we present a case of de novo leukemic variant of MCL with KIT D816V; to our knowledge, this...

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Highly Aggressive de novo Aleukemic Variant of Mast Cell Leukemia Without KIT D816V Mutation

Dear Editor, Mastocytosis refers to a group of rare clinical disorders, in which mast cells expand abnormally and accumulate in various organs [1, 2]. Mast cell leukemia (MCL) is a rare and aggressive form of systemic mastocytosis and accounts for 1% of all mastocytosis [3]. Due to its rarity, making a prompt diagnosis is difficult when unaware of the morphological characters of the abnormal ma...

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Activity of the tyrosine kinase inhibitor PKC412 in a patient with mast cell leukemia with the D816V KIT mutation.

The majority of patients with systemic mast cell disease express the imatinib-resistant Asp816Val (D816V) mutation in the KIT receptor tyrosine kinase. Limited treatment options exist for aggressive systemic mastocytosis (ASM) and mast cell leukemia (MCL). We evaluated whether PKC412, a small-molecule inhibitor of KIT with a different chemical structure from imatinib, may have therapeutic use i...

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Structure-based de novo design and identification of D816V mutant-selective c-KIT inhibitors.

To identify potent and selective inhibitors of D816V, the most common gain-of-function c-KIT mutant, we carried out structure-based de novo design using 7-azaindole as the core and the scoring function improved by implementing an accurate solvation free energy term. This approach led to the identification of new c-KIT inhibitors specific for the D816V mutant. The 3-(3,4-dimethoxyphenyl)-7-azain...

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Aleukemic variant of mast cell leukemia.

A woman was admitted with fatigue, icterus, thrombocytopenia (77 109/L), normocytic anemia (9.8 g/dL), and a normal white blood cell count (8.5 109/L). The peripheral blood smear showed metamyelocytes, myelocytes, monocytes, erythroblasts, and atypical cells with bilobulated nuclei resembling basophil granulocytes (left panel of figure). In the bone marrow there was a nearly complete infiltrati...

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ژورنال

عنوان ژورنال: Annals of Laboratory Medicine

سال: 2015

ISSN: 2234-3806,2234-3814

DOI: 10.3343/alm.2015.35.2.260