Juvenile myelomonocytic leukemia (JMML) is a rare clonal myeloproliferative disorder (MPD) of early childhood [1]. The median age at diagnosis is 2 years [1]. There is a male predominance with a male:female ratio of 2:1. Pallor, fever, infection, skin bleeding and cough are the most common presenting symptoms. Typically, there is marked hepatosplenomegaly. JMML rarely involves the central nervo...

A case of a localized testicular abscess due to Nocardia asteroides in a patient on immunosuppressive therapy for a myeloproliferative disorder is reported. Subsequent fatal dissemination of the infection to the prostate, lungs, and liver occurred. This represents the second reported case of nocardiosis of the testis. Extrapulmonary forms of nocardiosis must be recognized so that appropriate tr...

Down's syndrome (DS) is associated with duodenal atresia (DA) in about 8-10% of cases. Transient Myeloproliferative Disorder (TMD)/Acute Myeloid Leukemia (AML) is also associated with the trisomy 21 mutation. The occurrence of the two conditions together complicates the diagnosis and surgical management of the DA. We discuss the technical aspects of management of the DA in this clinical setting.

BACKGROUND AND OBJECTIVE Approximately 15% of patients with cancer will experience a thrombotic episode at some time. Some patients are at particularly high risk depending on the histology of the malignant disease. The aim of the study was to determine the actual prevalence of thrombotic episodes in oncohematologic patients. DESIGN AND METHODS We conducted a retrospective cohort analysis on a...

Oncogenic ras alleles are among the most common mutations found in patients with acute myeloid leukemia (AML). Previously, the role of oncogenic ras in cancer was assessed in model systems overexpressing oncogenic ras from heterologous promoters. However, there is increasing evidence that subtle differences in gene dosage and regulation of gene expression from endogenous promoters play critical...

Gelatinous bone marrow transformation (GMT) is a rare disorder characterized by the presence of fat cell atrophy, loss of hematopoietic cells, and deposition of extracellular gelatinous materials. GMT is not a specific disease, but is strongly associated with malnutrition and drugs. Albeit extremely rare, GMT has been reported in patients with myeloproliferative disorders. Herein, we report the...

Myeloproliferative neoplasms constitute a group of hematopoietic neoplasms at the myeloid stem cell level. Although mutations in the receptor tyrosine kinase KIT have been identified in patients with myeloproliferative neoplasm, the functional causality is unknown because of a lack of animal models. Here, we describe a mouse strain harboring a point mutation in the first Ig-like domain of Kit. ...

In this issue of Blood, Tiedt and colleagues use transgenic and retroviral models to demonstrate that the expression level of JAK2V617F plays an important role in determining myeloproliferative disease (MPD) phenotype.