Denosumab versus zoledronic acid for patients with beta-thalassemia major-induced osteoporosis.

ABSTRACT: The main aim of this study is to compare the 2 medications denosumab and zoledronic acid for patients with beta-thalassemia major induced osteoporosis. Patients with B-thalassemia major induced osteoporosis will undergo baseline assessment of the bone densitometry by bone density(DEXA) scan as a standard of care by the radiology department, then a blood test for bone-specific alkaline phosphatase and type-1 collagen telopeptide will be measured by the chemistry laboratory.Patients with B-thalassemia major induced osteoporosis, who are 18 years of age or more and willing to participate in the study will be enrolled after consenting by the primary investigator in hematology outpatient clinics. Patients with osteoporosis will receive 1 of the 2 medications; at the end of the year, DEXA scan will be done to compare the response of the 2 medications. The potential risks include drug-related side effects.The outcome will be measured biochemically by measuring bone-specific alkaline phosphatase and type 1 collagen carboxy telopeptide and radiologically by DEXA scan at baseline and 1 year using Z score.

A Case of Acquired von Willebrand Disease Secondary to Myeloproliferative Neoplasm.

Acquired von Willebrand Disease (AVWD) is a rare disorder in which qualitative or quantitative defects in von Willebrand factor (VWF) occur secondary to other conditions. AVWD occurs in patients with myeloproliferative disorders due to formation of autoantibodies against VWF and development of excessive shear stress causing disruption of VWF multimers. AVWD is different from congenital VWD in its acute onset and absence of family history. We report a 42-year-old gentleman with essential thrombocythemia, who was on cytoreductive therapy with hydroxyurea, and presented with an acute history of gum bleeding with hemoptysis, without any antecedent trauma or infections. His platelet count was very high, and prothrombin time and activated partial thromboplastin time were prolonged. The VWF ristocetin cofactor assay (VWF: RCo) was low, but VWF antigen level (VWF: Ag) was normal. Their ratio (VWF: RCo/VWF: Ag) was much lower than the acceptable lower limit. Treatment in AVWD is focused on addressing the underlying disorder. Early recognition of AVWD and its primary cause is mandatory in providing adequate therapy and achieving a cure.

Graves' Disease-Related Pancytopenia Improved after Radioactive Iodine Ablation.

Graves' disease is an autoimmune disease that affects the thyroid glands which often results in enlarged thyroid glands, and it is the most common cause of clinical hyperthyroidism especially in young patients. Radioiodine ablation is a radiation therapy in which radioactive iodine is administered to destroy or ablate thyroid cells. It is commonly used for the treatment of Graves' disease. We report on a 39-year-old male, who presented with Graves' disease, found to have pancytopenia and hypocellular bone marrow. Pancytopenia is a rare complication of thyrotoxicosis that is usually not severe and does not require supportive blood product transfusions. Our patient was treated with antithyroid medications followed by radioactive iodine ablation followed by a spontaneous recovery of pancytopenia.