An 82 year-old male with multiple myeloma comes to the emergency room with fatigue and weakness. His hemoglobin is 6.5 g/dL. He receives a blood transfusion and within 30 minutes after the transfusion is started, he develops burning at the infusion site, fever,chills, chest tightness, backache, headache, flank pain and reddish-colored urine. His temperature is 101 ⁰ F, heart rate is 94 beats per minute, respiratory rate is 26 breaths per minute, and blood pressure is 80/55 mm Hg. On physical examination, you see flushing on his face and hear wheezing in the lungs. Laboratory tests reveal hemoglobin 5.9 g/dL, free hemoglobin in urine, decreased serum haptoglobin, increased lactate dehydrogenase, increased indirect bilirubin level and positive direct antigen (Coombs) test. What is the underlying mechanism of this disorder?
A 32-year-old woman presents to the emergency department with a chief complaint of acute onset of shortness of breath. It started with a right-sided chest pain an hour ago. She denies fever, chills, cough, vomitings and diarrhea. She also has right lower extremity swelling that started two days ago, when she returned from Honolulu, Hawaii to State College, Pennsylvania traveling in long flights. The family history is notable for a father who died of an embolic stroke. She smokes one pack of cigarettes a day and uses oral contraceptives for birth control. On physical examination, she appears anxious and in respiratory distress. Her temperature is 99 °F (37.2 °C), heart rate is 108 beats/min, blood pressure is 165/105 mm Hg, respirations are 34 breaths/min, and oxygen saturation is 94% on room air. Chest x-ray film is normal. Ventilation-perfusion scan revealed a high probability of pulmonary embolism. She was initiated on heparin for anticoagulation. She requested early discharge from the hospital because of a family emergency and was transitioned to warfarin 5 mg once daily. Three days later, she developed pain and redness on her legs followed by a skin rash with large, irregular bullae with sharp red borders. Later, the skin necrosed and formed an eschar. Which of the following is a risk factor for pulmonary embolism in this patient?
Of the following, which is the most characteristic clinical feature of heparin-induced thrombocytopenia?
A 69 year-old female was admitted to the hospital and had undergone elective cardiac surgery for aortic valve replacement. She was administered heparin for the prevention of thrombosis. On the second day of her admission, she developed a urinary tract infection and was started on trimethoprim-sulfamethoxazole. On the sixth day of her hospital stay, she developed fever, chills, hypertension, tachycardia, chest pain and shortness of breath. On physical examination, you noticed pain, redness, and swelling of her right arm. A severe rash developed in the skin where heparin was given. The attending nurse reports that she is meticulously administering heparin via intravenous route and using heparin flushes before using intravenous lines. Ultrasound of the right arm revealed deep venous thrombosis and a CT angiogram revealed a blood clot in the right lung. Laboratory tests reveal the following: Erythrocyte count (RBC) 4.2 million/mm³ Male: 4.3 – 5.9 million/mm³ Female: 3.5 – 5.5 million/mm³ Erythrocyte sedimentation rate (Westergren) 12 mm/h Male: 0-15 mm/h Female:0-20 mm/h Hematocrit 39% Male:41% – 53% Female: 36% – 46% Hemoglobin, blood 13.4 g/dL Male:13.5-17.5 g/dL Female 12.0 – 16.0 g/dL Leukocyte count (WBC) 7200 /mm³ 4500-11,000/mm³ Neutrophils, segmented 56% 54% – 62% Neutrophils, bands 4% 3% – 5% Eosinophils 1% 1% – 3% Basophils 0.5% 0% – 0.75% Lymphocytes 28% 25% – 33% Monocytes 4% 3% – 7% CD4+ T-lymphocyte count 1100 /mm³ ≥500/mm³ Platelet count 40,000/mm³ (on the day of admission: platelet count was 340,000/mm³ 150,000 – 400,000/mm³ Reticulocyte count 1.0% 0.5% – 1.5% D-Dimer 450 ng/mL ≤250 ng/mL Partial thromboplastin time (aPTT) (activated) 27 seconds 25-40 seconds Prothrombin time (PT) 10 seconds 11-15 seconds Mean corpuscular hemoglobin (MCH) 29 pg/cell 25 – 35 pg/cell Mean corpuscular hemoglobin concentration (MCHC) 34% Hb/cell 31% – 36% Hb/cell Mean corpuscular volume (MCV) 94 μ m³ 80-100 μ m³ 4T score 7 PF4 ELISA Test Positive What is the next best step in the management of this patient?
John is a 17 year-old male who comes to your office for the establishment of his primary care. He reports a family history of von Willebrand disease. Of the following, which best explains the pathophysiology of von Willebrand disease?
A mother brings her 6 year-old boy to the emergency room with a swollen left knee joint. He denies a history of trauma to the knee. He reports similar swellings in the right knee joint, right and left elbow joints. Many times he feels the bleeding before he notices the swelling. For the last few days, he has been passing very red colored urine. In the physical examination, you notice a warm, swollen, erythematous left knee joint with severe effusion. There is sensory loss over the lateral and anterior thigh. The quadriceps muscle is weak and small compared to the muscle on his right lower extremity. Patellar reflex is absent. Upon direct questioning, the mother reports that her brother suffers from a bleeding disorder, with frequent nosebleeds. Many of her relatives also have similar bleeding disorders, affecting mostly males. Laboratory tests reveal a normal bleeding time, a normal prothrombin time but a prolonged PTT. As you review the labs with the patient, he developed a severe headache, unrelenting vomiting, and slurred speech. A gross physical examination showed focal neurologic deficits. What is the next best step in the management of this patient?
A mother brings her 7 year-old boy to the emergency room with a swollen left knee joint. He denies a history of trauma to the knee. He reports similar swellings in the right knee joint, right and left elbow joints. Many times he feels the bleeding before he notices the swelling. For the last few days, he has been passing very red colored urine. In the physical examination, you notice a warm, swollen, erythematous left knee joint with severe effusion. There is sensory loss over the lateral and anterior thigh. The quadriceps muscle is weak and small compared to the muscle on his right lower extremity. Patellar reflex is absent. Upon direct questioning, the mother reports that her brother suffers from a bleeding disorder, with frequent nosebleeds. Many of her relatives also have similar bleeding disorders, affecting mostly males. Laboratory tests reveal a normal bleeding time, a normal prothrombin time but a prolonged PTT. As you review the labs with the patient, he developed a severe headache, unrelenting vomiting, and slurred speech. A gross physical examination showed focal neurologic deficits. What is the most likely diagnosis in this patient?
A 61-year-old man presented with complaints of fatigue, headaches, and itching. Physical examination revealed a ruddy complexion and splenomegaly. Laboratory tests showed an elevated hematocrit, hemoglobin, and red blood cell count. A bone marrow biopsy confirmed polycythemia vera, a myeloproliferative disorder characterized by overproduction of red blood cells. Of the following, which is the treatment of choice in the management of polycythemia vera?
Abhimelech, a 58-year-old male, comes to the clinic reporting headaches, dizziness, and pruritus, particularly after warm baths. Upon examination, he appears plethoric, with a ruddy complexion. His medical history includes hypertension, managed with medication, but no other significant conditions. Laboratory tests reveal an elevated hemoglobin level of 18.5 g/dL, hematocrit of 57%, and red blood cell count of 6.2 million per microliter, leading to a suspicion of polycythemia. Further tests confirm low erythropoietin levels, normal oxygen saturation, and the presence of the JAK2 V617F mutation, which is pathognomonic for polycythemia vera (PV). Of the following, which vitamin levels are strikingly elevated in patients with polycythemia vera?
