Chronic myeloproliferative neoplasms (MPN) are clonal disorders of hematopoietic stem/progenitor cell characterized by thrombohemorrhagic complications and a tendency to transform into acute leukemia. The pathogenesis of thrombosis in MPN is complex and results from a multifaceted interplay of clinical and disease-related factors. Rotational thromboelastometry (ROTEM) provides the complete and rapid information about all stages of the coagulation process. Here, we assess ROTEM parameters as a screening of coagulation profile in patients with MPNs. In particular, higher mean maximum clot firmness values were found in Essential thrombocythemia and Polycythemia vera patients when compared to healthy controls. Rotational thromboelastometry may be able to detect MPN patients who are susceptible to thrombotic and/or hemorrhagic complications. The predictive value of ROTEM for thrombosis remains to be established to classify subsets of patients at prominent risk who may benefit from prophylaxis with antithrombotic drugs.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare subtype of AML characterized by the clonal proliferation of precursors of plasmacytoid dendritic cells. It presents with an aggressive behavior. The clinical findings include cytopenia, particularly thrombocytopenia. Although it responds well to chemotherapy initially, the relapse is a rule and prognosis is very poor. There is limited data published in the literature, making it very problematic to define the biological and clinical features, hence, the appropriate therapeutic approach. There are various treatment methods such as multiagent chemotherapy based on ALL or AML and/or hematopoietic stem cell transplantation. However, none of them is approved as a standard therapy. From this point of view, we herein report a 20-year-old case at onset of a leukemic form of BPDCN who survived 48 months after autologous hematopoietic stem cell transplantation.
Extramedullary relapse is an uncommon complication of acute promyelocytic leukaemia (APL). The most common site of extramedullary relapse is the central nervous system (CNS), and the majority of CNS relapses occur in patients with high-risk disease in which white blood cell count at presentation is greater than 10×103/μL. The best management of such patients is still controversial. We describe a 47-year-old man with APL who developed two CNS relapses which were diagnosed through the presence of t(15;17)(q22;q21) on PCR of the cerebrospinal fluid (CSF), despite presenting initially with intermediate-risk disease. We conclude that the intermediate risk group is very heterogeneous and these patients sometimes may behave like high-risk patients. Also, clinicians should take into account symptoms that can be related to CNS relapse in patients with APL and consider lumbar puncture even if radiological imaging does not reveal anything.