Incidence of pulmonary hypertension in patients with chronic myeloproliferative disorders.

STUDY OBJECTIVE: To assess the incidence of pulmonary hypertension (PH) in patients with chronic myeloproliferative disorders (CMPD). METHOD: Twenty-seven patients with a diagnosis of CMPD were included in the study. Patients were excluded if they had a secondary cause of PH. Diagnosis of PH was established if right ventricular systolic pressure (RVSP) by transthoracic echocardiography (TTE) was >35 mmHg. RESULTS: Diagnosis of PH was established in 14 out of 27 patients. Two patients were excluded from analysis because of poor ejection fraction on TTE, resulting in a final diagnosis of PH in 12 of 25 (48%) patients. Of these 25 patients, seven of nine with essential thrombocytosis (ET), five of 14 with polycythemia vera (PV), and 0 out of two with chronic myeloid leukemia (CML) had PH. All patients were asymptomatic at the time of their most recent visit. There was no relationship between PH and age at diagnosis, duration of disease, platelet count and hematocrit at diagnosis or during follow-up, both for the entire cohort or for specific diagnosis of ET or PV. CONCLUSION: Pulmonary hypertension appears to be common in patients with CMPD. Further studies are needed to evaluate the impact of treatment on PH and long-term survival in these patients.

Severe pulmonary complications in African-American patient after bortezomib therapy.

Bortezomib is a proteosome inhibitor with good clinical activity in multiple myeloma. Frequently described side effects are gastrointestinal symptoms, neuropathy, and thrombocytopenia. Even though pneumonia is listed as an infrequent toxicity, severe pneumonitis leading to respiratory distress had not been described until recently. This report was from a single institution in Japan. All these patients had received bone marrow transplant before therapy with bortezomib. To the authors knowledge, this is the first report of life-threatening pulmonary toxicity after bortezomib in a non-Japanese patient and without history of prior autologous peripheral stem cell transplant.

Cat scratch disease mimicking Richter's Syndrome in a patient with chronic lymphocytic leukemia.

Richter's Syndrome is a highly refractory and usually fatal condition. It occurs as a result of transformation of chronic lymphocytic leukemia (CLL) or low grade lymphoma into highly aggressive lymphoma. Patients usually present with rapidly enlarging lymph nodes and systemic symptoms like night sweats, fever and weight loss. We are reporting a case of CLL presenting with similar symptoms. Initial suspicion of Richter's Syndrome proved wrong when lymph node biopsy did not reveal evidence of high grade lymphoma. Instead it showed findings consistent with cat scratch disease (CSD), later confirmed by serology. To our knowledge this is the first reported case of CSD in a patient with CLL.