Systemic Mastocytosis with Associated Hematological Neoplasms. Diagnostic features and unique response pattern to tyrosine kinase inhibitors and allo-bone marrow transplantation therapy / Mastocitosis sistémica con neoplasias hematológicas asociadas. Características diagnósticas y patrón de respuesta único a los inhibidores de la tirosina cinasa y al tratamiento de trasplante de médula ósea

Systemic Mastocytosis is a clonal proliferation of mast cells; in a significant fraction of cases it is associated with another concurrent hematological neoplasm. Molecular analysis of KIT mutations and other associated genetic alterations suggest a common origin in the stem cell compartment. Mast cell infiltration patterns in bone marrow biopsy may be subtle in cases associated with t (8;21) AML. Here we report three cases of clonally related SM-AHN, two cases with SM-CMML and one case with SM- t (8;21) AML. We describe in detail the bone marrow infiltration pattern at diagnosis and during the course of treatment with allogeneic stem cell transplant and novel TK inhibitors, showing the unique dynamics of mast cell clearance after therapy.(AU)

La mastocitosis sistémica es una proliferación clonal de mastocitos que puede asociarse con otra neoplasia hematológica concurrente en una fracción significativa de los casos. El análisis molecular de mutaciones de KIT y otras alteraciones genéticas asociadas indican un origen común en el compartimento de células madre. Los patrones de infiltración de mastocitos en la biopsia de médula ósea pueden ser sutiles en los casos asociados con AML t (8; 21). Aquí se describen 3 casos de MS-NHA, 2 casos con MS-LMMC y un caso con MS-t (8; 21) LMA. Describimos en detalle el patrón de infiltración de la médula ósea en el momento del diagnóstico y durante el curso del tratamiento con alotrasplante de células madre y nuevos inhibidores de TK, mostrando la dinámica de la depuración de mastocitos después de la terapia.(AU)

Systemic Mastocytosis with Associated Hematological Neoplasms. Diagnostic features and unique response pattern to tyrosine kinase inhibitors and allo-bone marrow transplantation therapy.

Systemic Mastocytosis is a clonal proliferation of mast cells; in a significant fraction of cases it is associated with another concurrent hematological neoplasm. Molecular analysis of KIT mutations and other associated genetic alterations suggest a common origin in the stem cell compartment. Mast cell infiltration patterns in bone marrow biopsy may be subtle in cases associated with t (8;21) AML. Here we report three cases of clonally related SM-AHN, two cases with SM-CMML and one case with SM- t (8;21) AML. We describe in detail the bone marrow infiltration pattern at diagnosis and during the course of treatment with allogeneic stem cell transplant and novel TK inhibitors, showing the unique dynamics of mast cell clearance after therapy.