A 74 year-old man presents to your office because he is concerned about a small lump in the right axilla. He reports ‘I am fine otherwise’. He denies any fatigue, fever, night sweats or weight loss. Physical examination is significant only for a palpable small lymph node in the right axilla. You ordered a complete blood count which is significant for a white blood cell count of 94,300 /mm³. Peripheral smear shows lymphocytosis with many smudge cells. Of the following, what is the next best step in the management of this patient?

A 78 year-old man presents to your office and reports fatigue, fever, weight loss and night sweats for the last few months. He ignored his symptoms for a while but his wife insisted he must go to the doctor. Two weeks ago he had an insect bite and his ‘skin reacted badly like never before’. Physical examination is significant for cervical, axillary and inguinal lymphadenopathy. Abdominal examination found hepatomegaly and splenomegaly. Which of the following is the most likely diagnosis in this patient?

A 55-year-old man presents to your oncology office and reports feeling very tired, feeling short of breath and losing weight for the last 3 weeks. Upon review of his history, you note that he also has fevers, bruising easily and bone pains on various locations in his body. Upon physical examination, you note that he is pale with a petechial rash and that he has an enlarged liver and spleen. You note a wound on his right foot, which has not healed for over two months. You sent him for some laboratory tests, which reveal the following: Test Result Normal range Leukocyte count (WBC) 105,000 /mm³ 4500-11,000/mm³ Platelets 75,000 /mm³ 150,000 – 400,000/mm³ Hemoglobin 7.2 g/dL Male:13.5-17.5 g/dL Female 12.0 – 16.0 g/dL Blasts 80% <5% Hematocrit 20% Male:41% - 53% Female: 36% - 46% Peripheral smear showed pancytopenia; some cells have rod-shaped; needle-like, cytoplasmic, azurophilic granules. Further cytogenetic tests were done; Immunological phenotypes detected: MPO, CD11b, CD13, CD15, CD33, CD117, HLA-DR positive myeloblasts. The blasts react with antibodies to myeloperoxidase and antibodies to CD13, CD33, and CD34. Of the following, which is the most likely diagnosis in this patient?

A concerned mother brings her 6 year-old son to your office. She tells you that over the past 3 months the boy has been having severe fatigue, easy bruising, recurrent infections, fever, night sweats, and unintentional weight loss. He has had four spontaneous nose bleeds in the last two weeks alone. He was born in an Amish family when his mother was 45-years old in Belleville, a rural town in Pennsylvania, USA. His family history is significant for genetic disorders. You notice that the boy has a pale flat face, wide-set eyes with white spots on the periphery of iris, epicanthal folds, a single palmar crease across each hand. Pharynx is normal with no tonsillar exudate. On cardiac auscultation, you hear a holosystolic murmur that is consistent with a ventricular septal defect. Other physical examination findings include hepatomegaly, splenomegaly, lymphadenopathy, and a petechial rash over his entire body. Of the following, which is the most likely diagnosis in this patient?

A 29 year-old presents to your office and reports that she was frightened this morning when she used the bathroom because ‘ she passed blood instead of urine’. Further questioning reveals she passed reddish-brown urine. For the last few weeks she has been experiencing headaches, trouble breathing, back pain, and palpitations. On physical examination, she appears somewhat pale, with normal vital signs.You ordered some laboratory tests to find the source of her symptoms. Her white blood cells and platelet counts are decreased and serum LDH is elevated. Peripheral blood film showed anisocytosis, anisochromia and poikilocytosis with notable ovalocytosis and spherocytosis. Bone marrow studies revealed cellular activity with increased erythropoiesis and marrow macrophages laden with iron-laden particles. Urinary sediment stained with Prussian blue showed intensely positive reactions reflecting urinary iron loss as a result of intravascular hemolysis. You suspected paroxysmal nocturnal hemoglobinuria in this patient. What is the primary cause of death in patients with paroxysmal nocturnal hemoglobinuria?

A 38 year-old presents to your office and reports that she was frightened this morning when she used the bathroom because ‘ she passed blood instead of urine’. Further questioning reveals she passed reddish-brown urine. For the last few weeks she has been experiencing headaches, trouble breathing, back pain, and palpitations. On physical examination, she appears somewhat pale, with normal vital signs.You ordered some laboratory tests to find the source of her symptoms. Her white blood cells and platelet counts are decreased and serum LDH is elevated. Peripheral blood film showed anisocytosis, anisochromia and poikilocytosis with notable ovalocytosis and spherocytosis. Bone marrow studies revealed cellular activity with  increased erythropoiesis and marrow macrophages laden with iron-laden particles. Urinary sediment stained with Prussian blue showed intensely positive reactions reflecting urinary iron loss as a result of intravascular hemolysis. You suspected paroxysmal nocturnal hemoglobinuria in this patient and decided to use eculizumab. Of the following, which is true regarding eculizumab? 

A 44 year-old presents to your office and reports that she was frightened this morning when she used the bathroom because ‘ she passed blood instead of urine’. Further questioning reveals she passed reddish-brown urine. For the last few weeks she has been experiencing headaches, trouble breathing, back pain, and palpitations. On physical examination, she appears somewhat pale, with normal vital signs.You ordered some laboratory tests to find the source of her symptoms. Her white blood cells and platelet counts are decreased and serum LDH is elevated. Peripheral blood film showed anisocytosis, anisochromia and poikilocytosis with notable ovalocytosis and spherocytosis. Bone marrow studies revealed cellular activity with  increased erythropoiesis and marrow macrophages laden with iron-laden particles. Urinary sediment stained with Prussian blue showed intensely positive reactions reflecting urinary iron loss as a result of intravascular hemolysis. You suspect paroxysmal nocturnal hemoglobinuria.  Of the following, which best explains the etiology of paroxysmal nocturnal hemoglobinuria? 

A 34 year-old presents to your office and reports that she was frightened this morning when she used the bathroom because ‘ she passed blood instead of urine’. Further questioning reveals she passed reddish-brown urine. For the last few weeks she has been experiencing headaches, trouble breathing, back pain, and palpitations. On physical examination, she appears somewhat pale, with normal vital signs.You ordered some laboratory tests to find the source of her symptoms. Her white blood cells and platelet counts are decreased and serum LDH is elevated. Peripheral blood film showed anisocytosis, anisochromia and poikilocytosis with notable ovalocytosis and spherocytosis. Bone marrow studies revealed cellular activity with increased erythropoiesis and marrow macrophages laden with iron-laden particles. Urinary sediment stained with Prussian blue showed intensely positive reactions reflecting urinary iron loss as a result of intravascular hemolysis. You suspected paroxysmal nocturnal hemoglobinuria in this patient. Of the following, which is the gold standard test for diagnosing this disorder?

A 34 year-old presents to your office and reports that she was frightened this morning when she used the bathroom because ‘ she passed blood instead of urine’. Further questioning reveals she passed reddish-brown urine. For the last few weeks she has been experiencing headaches, trouble breathing, back pain, and palpitations. On physical examination, she appears somewhat pale, with normal vital signs.You ordered some laboratory tests to find the source of her symptoms. Her white blood cells and platelet counts are decreased and serum LDH is elevated. Peripheral blood film showed anisocytosis, anisochromia and poikilocytosis with notable ovalocytosis and spherocytosis. Bone marrow studies revealed cellular activity with  increased erythropoiesis and marrow macrophages laden with iron-laden particles. Urinary sediment stained with Prussian blue showed intensely positive reactions reflecting urinary iron loss as a result of intravascular hemolysis. Acidified serum (Ham) test came back as a positive test.  Most symptoms in this disease are due to which of the following? 

A 34 year-old male comes to your office with fever, chills, headache, fatigue, myalgias, arthralgia, anorexia, nausea, sweats,and depression. He returned from a hunting trip after spending two weeks in St.Paul, Minnesota. He reports that he is not sure but he might have had some tick bites during this trip. His past medication history is significant for splenectomy after a motor vehicle accident four years ago. Physical examination is significant for tenderness in the right upper quadrant. Laboratory tests showed anemia, thrombocytopenia, elevated transaminases and  intraerythrocytic ring-shaped parasites in tetrads on Giemsa-stained blood smears and later confirmed by a PCR assay which showed the DNA of the organism.     The patient was started on a 7 day course of quinine and clindamycin. A few days later, the patient called your office reporting tinnitus, headache, nausea, vomiting, diarrhea, abdominal pain, dizziness, flushing, visual disturbances, chest fluttering and shortness of breath. Physical examination showed fixed, dilated pupils. Fundoscopy revealed retinal artery spasm, disc pallor, and macular edema. ECG showed QT prolongation. What is the next best step in the management of this patient?