Venous thromboembolism (VTE) complicates 1 to 2 of 1000 pregnancies, with pulmonary embolism being a leading cause of maternal mortality and deep vein thrombosis an important cause of maternal morbidity, also on the long term. However, a strong evidence base for the management of pregnancy-related VTE is missing. Management is not standardized between physicians, centers, and countries. The man...

Common variable immunodeficiency is a rare immune deficiency, characterized by low levels of serum immunoglobulin G, A, and/or M with loss of antibody production. The diagnosis is most commonly made in adults between the ages of 20 and 40 years, but both children and older adults can be found to have this immune defect. The range of clinical manifestations is broad, including acute and chronic ...

Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by thrombocytopenia andmicroangiopathic hemolytic anemia (MAHA) without an obvious cause, and may include fever, mild renal failure, and neurologic deficits. It is characterized by a deficiency of the von Willebrand factor (VWF) cleaving enzyme, ADAMTS13 (a disintegrin and metalloproteinase, with a thrombospondin type 1 motif, ...

Survival in severe aplastic anemia (SAA) has markedly improved in the past 4 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive biologics and drugs, and supportive care. However, management of SAA patients remains challenging, both acutely in addressing the immediate consequences of pancytopenia and in the long term because of the disease’s natural history...

Our goal is to set forth our opinion of the best approach to managing adults with primary idiopathic (autoimmune) thrombocytopenic purpura (ITP), with emphasis on the word “opinion.” The paucity of evidence-based medicine on this topic has been noted.1 Both the American Society of Hematology2 and the British Committee for Standards in Haematology General Haematology Task Force3 have issued “pra...

I will take advantage of the history of two gentlemen that I encountered in the office some years apart to illustrate how I manage subjects referred for the investigation of erythrocytosis and are then found to have polycythemia vera (PV) rather than relative or secondary erythrocytosis (Table 1). Therefore, this manuscript is not intended to provide a thorough description of the disease or to ...

A debate among gynecologic and reproductive surgeons is whether or not there is a clinical need to treat all intramural myomas. Considerations include myoma size and number, ability to access them, whether or not they compromise the endometrium, and treatment effect on gynecologic, reproductive, and obstetric outcomes. We conducted a detailed study regarding intramural myomas, their prevalence ...

A 19-year-old woman with end-stage renal failure due to congenital kidney disease underwent a living-donor renal transplant in 2006. Eight years later, she was diagnosed with EBV MYC translocation– confirmed BL-PTLD stage IVB (Figure 1) while on treatment with mycophenolate mofetil (MMF), tacrolimus, and low-dose steroids. EBV viral load was very high (5.84 log IU/mL). Due to the aggressive pre...

Critical issues in determining therapeutic strategies for patients with myelodysplastic syndromes (MDSs), who usually die of bone marrow failure with or without conversion to acute myeloid leukemia (AML), include host factors in these mainly older patients, disease heterogeneity, lack of pathogenetic understanding, and a dearth of effective treatments. Even the issue of whether these clonal dis...

G optic neuritis is still somewhat of a management puzzle for ophthalmologists despite two excellent studies in recent years, says Anthony C. Arnold, MD, Professor of Ophthalmology, Chief of the Division of Neuro-Ophthalmology, and Director of the Optic Neuropathy Center at the Jules Stein Eye Institute. “Whether to treat typical monosymptomatic optic neuritis with steroids or not has always be...