Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T) and granulocytic sarcoma are two rare myeloid neoplasms. RARS-T is a Myelodysplastic/Myeloproliferative Neoplasm (MDS/MPN) characterized by overlapping myeloproliferative (platelets count higher than 450x109/L associated with proliferation of large atypical megakaryocytes similar than those observed in essen...

Myeloid sarcoma is an extramedullary tumor composed of immature myeloid cells that efface the underlying tissue architecture. It is usually associated with acute myelogenous leukemia, but can be associated with myeloproliferative neoplasms, myelodysplastic disorders, or myeloproliferative/ myelodysplastic syndromes. If it is unrecognized, appropriate chemotherapy may be delayed and survival jeo...

Bone marrow and peripheral blood cells from three newborns with Down's syndrome and transient myeloproliferative disorders were cultured in vitro. In the methylcellulose semiliquid system, normal colony formation with maturation and differentiation into granulocytes and monocyte-macrophages were observed in all three patients. This is different from the growth pattern usually seen in acute nonl...

The plasma of patients with myeloproliferative diseases was examined by polyethylene glycol (PEG) precipitation, analytical ultracentrifugation, and immunoaffinity chromatography for the presence of high molecular weight complexes of IgG and fibronectin. Abnormal circulating high molecular weight material was identified by ultracentrifugation in all patients. This was precipitated by PEG and wa...

Ruxolitinib is widely in use for treatment of myeloproliferative disorders. It causes inhibition of the Janus kinase (JAK) signal transducer and activation of transcription (STAT) pathway, which plays a key role in the underlying pathophysiology of myeloproliferative diseases. We describe a case of reactivation pulmonary tuberculosis in a retired physician while on treatment with ruxolitinib. W...

| 1634 | haematologica | 2009; 94(12) The World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues includes within myeloid neoplasms the category “Myelodysplastic/myeloproliferative neoplasms”. According to Vardiman et al., these are “clonal myeloid neoplasms that at the time of initial presentation have some clinical, laboratory or morphologic findings tha...

INTRODUCTION The 2008 World Health Organization classification of myeloid neoplasms includes the diagnostic category, myelodysplastic/myeloproliferative neoplasms (MDS/MPN), which encompasses those rare clonal myeloid proliferations that at initial presentation, show overlapping myeloproliferative and myelodysplastic features, making classification as either a myelodysplastic syndrome (MDS) or ...

JAK2 is a tyrosine kinase gene that plays an essential role in the development of normal haematopoiesis. Hyperactivation of JAK2 occurs in myeloproliferative neoplasms by different mechanisms. As a consequence, JAK2 inhibitors have been designed to suppress the cytokine signalling cascade caused by the constitutive activation of JAK2. In clinical trials, JAK2 inhibitors are efficient in decreas...

Haematological disorders are getting more and more molecularly defined. This is specially so in the field of haematological malignancies. The molecular understanding has helped in fine-tuning the diagnosis, prognosis and management. The tests are becoming widely available and they enjoy both sensitivity and specificity. Following is an overview of clinical applications of molecular haematology,...

Occurrences of second malignancies in hairy cell leukaemia are well recognised. Most of these malignancies are either solid tumours or lymphoproliferative disorders. The association of myeloproliferative disorders with hairy cell leukaemia (HCL) is very rare. This report describes a case of a patient with HCL who after remaining in remission developed Philadelphia chromosome positive chronic my...