Thelansis

  Juvenile Myelomonocytic Leukemia (JMML) is a type of pediatric myelodysplastic/myeloproliferative neoplasm characterized by persistent, excessive production of myeloid progenitors and monocytes, a severe clinical course, and unfavorable outcomes. The deregulation of the intracellular Ras signaling pathway, caused by mutations in one or rarely more than one of the five primary genes (PTPN11, NRAS, KRAS, NF1, or CBL), is the common molecular feature of JMML. At diagnosis, clinical signs include non-specific symptoms such as infections, fatigue, or growth failure, and splenomegaly is present in nearly all cases. Other symptoms may include hepatomegaly, lymphadenopathy, and pulmonary or intestinal infiltration. Cutaneous features may range from eczematous lesions to petechiae. JMML must be differentiated from other conditions, including infections, genetic or non-leukemic hematological disorders, and Ras-related autoimmune lymphoproliferative disease. Allogeneic hematopoietic stem ...

 Juvenile Myelomonocytic Leukemia (JMML) is a type of pediatric myelodysplastic/myeloproliferative neoplasm characterized by persistent, excessive production of myeloid progenitors and monocytes, a severe clinical course, and unfavorable outcomes. The deregulation of the intracellular Ras signaling pathway, caused by mutations in one or rarely more than one of the five primary genes (PTPN11, NRAS, KRAS, NF1, or CBL), is the common molecular feature of JMML. At diagnosis, clinical signs include non-specific symptoms such as infections, fatigue, or growth failure, and splenomegaly is present in nearly all cases. Other symptoms may include hepatomegaly, lymphadenopathy, and pulmonary or intestinal infiltration. Cutaneous features may range from eczematous lesions to petechiae. JMML must be differentiated from other conditions, including infections, genetic or non-leukemic hematological disorders, and Ras-related autoimmune lymphoproliferative disease. Allogeneic hematopoietic stem cel...