The utility of therapeutic plasma exchange in Hyperviscosity syndrome associated with juvenile rheumatoid arthritis: A case report.

Hyperviscosity syndrome (HVS) is a life-threatening syndrome caused by high concentrations of large plasma proteins like IgM, rheumatoid factor, and other immune complexes, leading to increased blood viscosity and symptoms such as visual abnormalities, neurological impairment, bleeding diathesis, and thrombosis. While Waldenström's macroglobulinemia accounts for 80% to 90% of cases, HVS may develop in other clinical settings characterized by elevations in plasma proteins. Limited evidence currently exists describing the safety and efficacy of therapeutic plasma exchange (TPE) for the management of HVS secondary to non-neoplastic conditions.

Bilateral Forearm Pseudotumors in an Adult with Hemophilia A and Ollier Disease: A Case Report.

Case: A 29-year-old man with mild hemophilia A and Ollier disease presented with bilateral atraumatic forearm pseudotumors. Both forearm pseudotumors were successfully treated surgically with staged radical extirpation and factor VIII replacement therapy.

Conclusion: Pseudotumors typically occur in adolescents with severe, poorly controlled hemophilia A.

IVIg for Treatment of Severe Refractory Heparin-Induced Thrombocytopenia.

Background: Heparin-induced thrombocytopenia (HIT) complicated by severe thrombocytopenia and thrombosis can pose significant treatment challenges. Use of alternative anticoagulants in this setting may increase bleeding risks, especially in patients who have a protracted disease course. Additional therapies are lacking in this severely affected patient population.

A case of Philadelphia chromosome positive myeloproliferative neoplasm in a pregnant woman with unusual primary myelofibrosis features.

Myeloproliferative neoplasms (MPNs) are traditionally separated into BCR-ABL-positive chronic myeloid leukemia (CML), and BCR-ABL-negative MPNs including primary myelofibrosis (PMF), essential thrombocythemia (ET), and so forth. One of the diagnostic requirements for PMF and ET is the absence of the Philadelphia chromosome, while its presence is almost universally indicative of CML. However, a diagnostic dilemma arises when Philadelphia chromosome-positive MPNs lack the majority of the typical features seen in CML.

Seventy-four-year-old woman with myelodysplastic syndrome and splenomegaly.

We present a 74-year-old woman with a recent diagnosis of myelodysplastic syndrome who presented with left upper quadrant abdominal pain and fatigue with significant splenomegaly, anemia, and thrombocytopenia. She underwent splenectomy and bone marrow biopsy. Pathology of both spleen and bone marrow revealed an unusual diagnosis.

Thrombotic thrombocytopenic purpura and adult onset Still's disease.

Background: Thrombotic thrombocytopenic purpura (TTP) has a high mortality rate if undiagnosed and untreated. Although recent literature supports the role of ADAMTS13 (a disintegrin-like metalloproteinase with thrombospondin type 1 repeats), the von Willebrand factor cleaving protease, in the pathogenesis of the disease, many aspects of the disease remain a mystery. Various drugs and autoimmune conditions, such as systemic lupus erythematosus and the antiphospholipid syndrome, have been observed in association with TTP.

Paraplegia as the presenting manifestation of extramedullary megakaryoblastic transformation of previously undiagnosed chronic myelogenous leukemia.

Extramedullary tumors, also known as granulocytic sarcomas (GS), occur most frequently in acute myelogenous leukemia (AML). They may signal the onset of the accelerated phase of chronic myelogenous leukemia (CML) or the blastic transformation of a myeloproliferative disorder. Occasionally, a GS may be the presenting sign of undiagnosed AML, and rarely the presenting sign of undiagnosed CML or aleukemic leukemia.

Blood bank on-call physician's experiences at a large university medical center.

Background: The responsibilities of the blood bank on-call physician (blood bank physician from here on) encompass many aspects of transfusion medicine and physician education. This physician is available 24 hours a day to address any issues concerning the collection and transfusion of blood and blood components. The purpose of this study was to identify and categorize the issues that may confront a blood bank physician.