Eltrombopag dose predictors in thrombocytopenic subjects with hepatitis C virus infection.

This study aims to identify patient characteristics that predict effective eltrombopag dosage for the treatment of Hepatitis C virus (HCV)-related thrombocytopenia. Demographic, clinical and genetic data collected from thrombocytopenic patients (n = 1463, age ≥ 18 years) with chronic HCV infection who were able to achieve a target platelet count of > 90 × 10(9) /L following eltrombopag treatment. Patients were categorized into four groups (25, 50, 75, and 100 mg) based on the eltrombopag dose needed to achieve the target platelet count. Eltrombopag dose predictors were identified using a two stage approach. First, bivariate analysis, using anova for continuous variables and Chi-square test for categorical variables, was performed to identify possible predictors of eltrombopag dose (P < 0.05). Second, ordinal logistic regression with stepwise addition followed by backward deletion was then performed using predictors identified in bivariate analysis step to produce final model containing independent predictors at P < 0.05. Ordinal logistic model identified several predictors of eltrombopag dose. Predictors of higher eltrombopag dose include: having a HCV genotype 2 or 3, being Central/South Asian, being White (Caucasian or European heritage), increased weight, and increased spleen length. Predictors of lower eltrombopag dose include: female gender, increased age, having a higher ALP plasma concentration, increased creatinine clearance, increased baseline lymphocytes count, and increased baseline platelet count. In conclusion, this study identified patient characteristics that predict effective eltrombopag dose for the treatment of HCV-related thrombocytopenia. Early selection of the optimal eltrombopag dose expedites the initiation of antiviral therapy. This is expected to improve the antiviral therapy outcome before the patient progress into liver decompensation.

Durable hematological and major cytogenetic response in a patient with isolated 20q deletion myelodysplastic syndrome treated with lenalidomide.

Myelodysplastic syndrome (MDS) is a clonal bone marrow disorder characterized by ineffective hematopoiesis. It is characterized by peripheral blood cytopenia and significant risk of progression to acute myeloid leukemia result. Deletion of the long arm of chromosome 20 (20q deletion) is present in 3-7% of patients with MDS. Lenalidomide is an immunomodulatory agent with antiangiogenic activity. It is FDA approved for the treatment of anemia in patients with low or int-1 risk MDS with chromosome 5q deletion with or without additional cytogenetic abnormalities. Study of lenalidomide in patients with MDS without 5q deletion but other karyotypic abnormalities demonstrated meaningful activity in transfusion dependent patients; however, response of patients with isolated 20q deletion to lenalidomide is not known. We are reporting a patient with 20q deletion MDS treated with lenalidomide after he failed to respond to azacytidine; to our knowledge this is the first report of a patient with isolated 20q deletion treated with lenalidomide.

Angioedema with normal C1q and C1 inhibitor: an atypical presentation of Waldenström macroglobulinemia.

Angioedema is a recurrent, non-pitting, non-pruritic, transitory swelling due to transient increase of endothelial permeability in the capillaries of the deep cutaneous and mucosal layers. Angioedema is generally categorized based on etiology, and characteristic lab findings are associated with each category. Cases of acquired angioedema associated with myeloproliferative disorders have been described in the literature, but these have been associated with a characteristic low C1q, a defining laboratory finding in acquired angioedema. Here we present a case of 68-year-old female with acquired angioedema that was not associated with low C1q, but was found to have Waldenström disease. Her angioedema responded dramatically to combination therapy consisting of bortezomib, rituximab, and dexamethasone.