First-line ibrutinib for Bing-Neel syndrome.

The authors present a case of an elderly man with a history of Waldenstrom macroglobulinaemia in remission who presented with progressively worsening gait abnormalities and falls for several months. His examination was notable for bilateral lower extremity weakness and an unsteady gait. Brain and spinal MRI showed focal leptomeningeal enhancement in the brain and spinal column.

Diffuse alveolar hemorrhage: retrospective review of clinical outcome in allogeneic transplant recipients treated with aminocaproic acid.

Diffuse alveolar hemorrhage (DAH) after allogeneic hematopoietic stem cell transplantation (HSCT) is often fatal. Standard therapy with high-dose corticosteroid is not always effective. There is paucity of data in the literature about other potentially useful agents, such as aminocaproic acid (Amicar) in the post-transplantation setting.

Hairy cell leukemia: an elusive but treatable disease.

Hairy cell leukemia (HCL) is a unique chronic lymphoproliferative disorder that can mimic or coexist with other clonal hematologic disorders and has been associated with autoimmune disorders. It should be entertained as an alternative diagnosis in patients with cytopenias being assigned the diagnosis of aplastic anemia, hypoplastic myelodysplastic syndrome, atypical chronic lymphocytic leukemia, B-prolymphocytic leukemia, or idiopathic myelofibrosis. Causative etiology or molecular defects remain unclear, although nonspecific chromosomal and molecular changes have been described.

Transfusion management in sickle cell disease.

Sickle cell disease (SCD) is the most commonly inherited hemoglobinopathy in the United States. Blood transfusion is a critical part of the multidisciplinary approach necessary in the management of SCD; however, blood transfusions are not without complications. The successful use of transfusion as a treatment strategy in SCD requires the critical review and knowledge of transfusion methods, generally accepted indications, clinical situations in which transfusion generally is not considered, the selection of blood products, and strategies to prevent transfusion-related complications.

Non-pharmacologic approaches to graft-versus-host prevention.

Allogeneic transplant offers a curative option for selected hematological malignancies and improved disease free survival in those where cure is not possible. For patients in these categories, Graft-versus-host disease (GVHD) occurs often and is a significant cause of morbidity and mortality following transplantation. Pharmacological immunosuppression has considerable toxicity and controls GVHD in about 75% of transplant recipients when used in combination and continually.