Massive Hemoptysis Due to the Rupture of Thoracic Aortic Aneurysm Caused by Leukemic Cell Infiltration in a Patient With Chronic Myelomonocytic Leukemia.

Hemoptysis is occasionally experienced in patients with hematological malignancies who have respiratory tract infection and severe thrombocytopenia. Thrombocytopenia due to hematological disease is one cause of hemoptysis. Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy characterized by both a myeloproliferative neoplasm and a myelodysplastic syndrome. This malignancy often infiltrates various extramedullary organs and has a poor prognosis. An 84-year-old Japanese man with CMML was suffered from hemoptysis and dyspnea. When he arrived at the emergency room, hemoptysis stopped. His white blood cell count was 866 × 109/L with 3.5% blast cells and 36.5% monocytes; hemoglobin was 6.7 g/dL; platelets count was 19 × 109/L; and C-reactive protein was 16.23 mg/dL. Chest X-ray examination revealed an invasion shadow near the mediastinum in the left upper lung field. Chest computed tomography revealed a tumorous lesion in the left upper lobe, which had progressed to the mediastinum and formed an infiltration shadow around it. He was administered the antibiotics and the hemostatic agents under hospitalization. He also received blood transfusion for anemia and thrombocytopenia. Rapid improvement in oxygenation was observed along with a rapid decrease in blood levels in the sputum. On the eighth days of hospitalization, however, the patient newly developed massive hemoptysis and died. Autopsy revealed rupture of a thoracic pseudoaneurysm due to infiltration of leukemia cells in the tunica media and lung. Clinicians should consider thoracic aortic aneurysms as a possible cause of hemoptysis even in cases with small hemoptysis. It should be noted that in CMML patients, direct infiltration of leukemia cells in the vascular wall can cause aneurysm formation.

Characteristics and prognosis of patients with non-immunoglobulin-M monoclonal gammopathy of undetermined significance: a retrospective study.

Non-immunoglobulin (Ig)-M monoclonal gammopathy of undetermined significance (MGUS) is a precursor lesion with the potential to evolve into a malignant plasma cell neoplasm. The prevalence of MGUS differs by ethnicity and is lower in the Japanese population than in the Western population. However, there is limited evidence about the clinical course of MGUS in Asian races. The present study aims at elucidating the clinical course and prognosis of Japanese patients with non-IgM MGUS in the clinical setting. We retrospectively examined 1009 patients with non-IgM MGUS identified by screening procedures. The median overall survival of these patients was > 20 years, and only one-fifth patients died of plasma cell neoplasms. The cumulative incidence of plasma cell neoplasms requiring treatment was 19%. Multivariate analysis revealed that immunoparesis and female gender were independent factors affecting treatment requirement. Although the characteristics and clinical course of patients with non-IgM MGUS obtained in this study were found to be essentially similar to those of previous studies, we report here for the first time that female gender is a significant independent factor for requiring treatment.

Multiple cystic lung lesions and autoimmune thrombocytopaenia developing after chemotherapy for pulmonary indolent B-cell lymphoma with plasmacytic differentiation.

Among lung malignancies, primary pulmonary lymphoma is rare and many of them are indolent B-cell lymphomas. We describe a case of primary pulmonary indolent B-cell lymphoma with plasmacytic differentiation, which exacerbated with the manifestation of macroglobulinaemia and was successfully treated using chemotherapy. The patient subsequently developed pulmonary cysts and thrombocytopaenia due to autoimmune pathology and was successfully treated using prednisolone. This case suggests that in indolent B-cell lymphoma with plasmacytic differentiation, immunoglobulin M level should be carefully followed even if it is within the normal range at lymphoma onset. Additionally, new cystic pulmonary infiltrates that develop during the post-treatment follow-up of an indolent pulmonary B-cell lymphoma may indicate pulmonary lymphoma recurrence, but there is also a possibility of an immunological complication.

Acquired von Willebrand Syndrome due to Aortic Valve Stenosis in a Case with Antiphospholipid Antibody.

Acquired von Willebrand syndrome (AVWS) is a bleeding disorder caused by an acquired deficiency of von Willebrand factor (vWF). Some patients with AVWS show a low bleeding tendency and are diagnosed by the presence of a mild prolongation of activated partial thromboplastin time (APTT) preoperatively. Another cause of APTT prolongation is the presence of antiphospholipid antibody (aPL). We experienced a case of AVWS due to aortic valve stenosis in a patient with aPL in whom aortic valve replacement surgery was successful with vWF replacement. In patients with AVWS-associated disorders who are identified based on APTT prolongation at the preoperative examination, both vWF and aPL screening tests must be performed.