|Coexistence of t(5;17)/NPM1-RARA and t(9;22)/BCR-ABL1 in chronic myeloid leukemia at initial diagnosis|
|Juvenile myelomonocytic leukemia: a surprising cause of peri-orbital tumor and squint|
|Oral ulceration: an unusual manifestation of lymphomatoid granulomatosis|
|Neutrophil faggot cells and inv(16): not such a fortuitous association?|
|Successful treatment of monomorphic epitheliotropic intestinal T cell lymphoma with pralatrexate|
|Extracavitary primary effusion lymphoma (PEL) presenting as bilateral adrenal gland involvement in an HIV-positive patient|
|Atypical bortezomib-induced neutrophilic dermatosis|
|RNA-binding protein ( RBFOX1 ) inherited polymorphism rs8051518 is not associated with splice factor mutations in myelodysplastic syndromes and myeloproliferative neoplasms|
|Lymphomatoid granulomatosis mimicking cancer and sarcoidosis
Two cases of misdiagnoses of lymphomatoid granulomatosis are discussed here. Lymphomatoid granulomatosis is an Epstein-Barr virus-associated lymphoproliferative disorder with aggressive behavior. Due to its rarity and many presentations, delay in diagnosis and treatment is common. Its histological features including large atypical B-cells, T cell predominance, angiocentricity, necrosis, and evidence of EBV-positive cells should elicit the diagnosis of lymphomatoid granulomatosis. The settings that are described here have not yet been described in the literature.
|Thromboembolic events in polycythemia vera
Thromboembolic events and cardiovascular disease are the most prevalent complications in patients with polycythemia vera (PV) compared with other myeloproliferative disorders and are the major cause of morbidity and mortality in this population. Moreover, a vascular complication such as arterial or venous thrombosis often leads to the diagnosis of PV. The highest rates of thrombosis typically occur shortly before or at diagnosis and decrease over time, probably due to the effects of treatment. Important risk factors include age (≥ 60 years old) and a history of thrombosis; elevated hematocrit and leukocytosis are also associated with an increased risk of thrombosis. The goal of therapy is to reduce the risk of thrombosis by controlling hematocrit to < 45%, a target associated with reduced rates of cardiovascular death and major thrombosis. Low-risk patients (< 60 years old with no history of thrombosis) are managed with phlebotomy and low-dose aspirin, whereas high-risk patients (≥ 60 years old and/or with a history of thrombosis) should be treated with cytoreductive agents. Interferon and ruxolitinib are considered second-line therapies for patients who are intolerant of or have an inadequate response to hydroxyurea, which is typically used as first-line therapy. In this review, we discuss factors associated with thrombosis and recent data on current treatments, including anticoagulation, highlighting the need for more controlled studies to determine the most effective cytoreductive therapies for reducing the risk of thrombosis in patients with PV.