A previously healthy 17-year-old boy is brought to the emergency department by his mother for further evaluation after elective removal of his wisdom teeth. During the procedure, the patient had persistent bleeding from the teeth's surrounding gums. Multiple gauze packs were applied with minimal effect. The patient has a history of easy bruising. The mother says her brother had similar problems when his wisdom teeth were removed, and that he also has a history of easy bruising and joint swelling. The patient takes no medications. His temperature is 37°C (98.6°F), pulse is 90/min, and blood pressure is 108/74 mm Hg. Laboratory studies show: Hematocrit 35% Leukocyte count 8,500/mm3 Platelet count 160,000/mm3 Prothrombin time 15 sec Partial thromboplastin time 60 sec Bleeding time 6 min Fibrin split products negative Serum Urea nitrogen 20 mg/dL Creatinine 1.0 mg/dL Bilirubin Total 1.0 mg/dL Direct 0.5 mg/dL Lactate dehydrogenase 90 U/L Peripheral blood smear shows normal-sized platelets. Which of the following is the most likely diagnosis?
AHemophilia
BBernard-Soulier syndrome
CGlanzmann thrombasthenia
DVon Willebrand disease
EImmune thrombocytopenia
A 38-year-old woman comes to the physician for a follow-up examination. She was diagnosed with immune thrombocytopenic purpura at the age of 37 years and has been treated with glucocorticoids and intravenous immune globulin. She has visited the emergency department 3 times in the past 4 months for nose bleeds, which required cauterization. Her platelet counts on her previous visits were 18,320/mm3, 17,500/mm3, and 19,100/mm3. Current medications include dexamethasone and a multivitamin. She has no children. Her immunizations are up-to-date. Vital signs are within normal limits. Examination shows petechiae on the bilateral lower extremities. Cardiopulmonary examination shows no abnormalities. The abdomen is soft and nontender; there is no organomegaly. Her hemoglobin concentration is 13.3 g/dL, leukocyte count is 8,100/mm3, and platelet count is 13,000/mm3. Her blood type is A negative. Serology for hepatitis C and HIV is negative. Which of the following is the most appropriate next step in management?
ASchedule splenectomy
BRomiplostim therapy
CRituximab therapy
DObservation and follow-up
EDanazol therapy
A 13-year-old girl is brought to the pediatrician due to a 4-month history of heavy vaginal bleeding during menstrual periods. She endorses episodes of bleeding gums after brushing her teeth and experienced prolonged bleeding after tonsillectomy 6 years ago. Her mother states that she bled significantly during childbirth and that the girl’s older brother has similar symptoms including easy bruising. Vitals were stable and physical exam was not revealing. Laboratory studies show: Platelet count: 72,000/mm^3 Bleeding time: 14 min Prothrombin time: 12 secs (INR = 1) Partial thromboplastin time: 40 secs Blood smear demonstrates increased megakaryocytes and enlarged platelets. Platelets do not aggregate to ristocetin. Which of the following is the most likely diagnosis?
AGlanzmann thrombasthenia
BIdiopathic thrombocytopenic purpura (ITP)
CBernard-Soulier syndrome
DAspirin or NSAID use
Evon Willebrand disease (vWD)
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