Heme/Onc
A 5 y/o child presents with lymphadenopathy, bone pain, bleeding, and fever. Bone marrow demonstrates > 20% lymphoblasts. What is the most likely diagnosis?
ALL
Dx: bone marrow w/ > 20% blasts
Tx: Highly responsive to chemotherapy (remission > 90%)
Mnemonic: Anemic, Lumpy, Limping
Which clotting factor does Von Willebrand prevent the degradation of?
Factor VIII
A patient has recurrent deep vein thromboses. Which of the following is the most likely diagnosis?
A. Alpha-thalassemia
B. Factor V Leiden
C. Hemophilia B
D. Von Willebrand disease
B. Factor V Leiden
*Factor V Leiden most common inherited hypercoagulability disorder
What is Eisenmenger syndrome?
Eisenmenger syndrome = left-to-right shunt → right-to-left shunt
*Progression to right-to-left shunting
-Right-to-left shunt causes deoxygenated blood from the right side of the heart enters the systemic circulation
(more in depth explanation on the document I'm going to post)
Patient presents with painless lymphadenopathy. CXR shows mediastinal adenopathy, an excisional biopsy of his lymph node is completed and shows reed-sternberg cells. What is the most likely diagnosis?
Tx: Chemo, radiation -- highly curable compared to Non-Hodgkin lymphoma
What are you most likely to see on blood smear for a patient diagnosed w/ AML/APL?
myeloblasts with Auer rods and > 20% blasts in blood smear
Tx: Chemo, bone barrow transplant
Mnemonic: A-Rod plays for the NY Yankees in the big APL
A 5yo boy presents to the clinic with recurrent bruising on his lower extremities. The patient’s guardian reports that the child often gets swollen and painful ankles that persist for days to weeks after minor falls. Lab evaluation reveals aPTT of 80 seconds (prolonged), a PT of 12 second (normal), and a platelet count of 180,000/mcL (normal). Deficiency in which of the following accounts for the suspected diagnosis?
A. Factor VIII
B. Factor X
C. Protein C
D. Vitamin K
A. Factor VIII
Dx. Hemophilia A
-Hemophilia A = 8, Hemophilia B = “Benign(9)"
-Hemophilia causes prolonged PTT, not PT
Hemarthrosis and easy bruising in young boy = Hemophilia
Tx: Factor VIII or DDAVP (Tx for Hem B is Factor IX, NOT DDAVP)
What is the triad of findings found in hemolytic uremic syndrome (HUS)?
Hemolytic anemia
Thrombocytopenia
Acute kidney injury → oliguria
*Usually occurs in young child after a GI illness
HUSS
Hamburger
Urinary symptoms
Shitting
School-aged
What are the 4 main components of Tetralogy of Fallot?
BONUS: Is it cyanotic or acyanotic?
Right ventricular outflow obstruction
Right ventricular hypertrophy
Overriding aorta
Ventricular septal defect
*You WILL get tested on this. Usually the question will describe some of the components, and you need to choose the other component that is missing.
*Mnemonic: PROVe
Pulmonic stenosis, Right ventricular hypertrophy, Overriding aorta, VSD
* Is it cyanotic or acyanotic? Cyanotic
Anything that starts with a T = cyanotic (think turquoise)
A 12-month-old boy presents to the clinic with his parents for a routine well-child check. Cardiac auscultation reveals a cardiac murmur. Which of the following qualities would indicate this is an innocent murmur?
A. Grade 3 intensity
B. Holosystolic timing
C. Increased intensity in the upright position
D. Vibratory quality
D. Vibratory quality
Which leukemia presents in older individuals and is diagnosed with smudge cells on peripheral smear?
CLL
Mnemonic: Crushed Little Lymphocytes = smudge cells
Tx: observation, if lymphocytes are > 100,000 or symptomatic --> chemo
A 9-year-old female is being evaluated for frequent epistaxis and easy bruising tendencies. Bleeding time is prolonged. PT, INR, and platelet count are within normal limits. PTT is slightly elevated. Which of the following is the most likely diagnosis?
A. Hemophilia A
B. Hemophilia B
C. Factor V Leiden
D. Von Willebrand disease
D. Von Willebrand Disease
*Von willebrand disease most common congenital bleeding disorder
*Hemophilia would present with intense bleeding and swelling of the joints; also Hemophilia most likely in males
A 26 yo female at 32 weeks gestation reports swelling and discomfort in her right leg. Ultrasound of the leg reveals a noncompressible vein. Which of the following treatments is contraindicated?
A. Inferior vena cava filter
B. Low molecular weight heparin
C. Unfractionated heparin
D. Warfarin
D. Warfarin
*Warfarin CI in pregnancy, teratogenicDx: DVT
Tx: unfractionated heparin or LMWH
A 3 day old baby girl has a holosystolic murmur along the left lower sternal border. What is the most likely diagnosis?
A. Aortic stenosis
B. Atrial septal defect
C. Patent ductus arteriosus
D. Ventricular septal defect
D. Ventricular septal defect (VSD)
*most common pathologic murmur in children
*loud, harsh, holosystolic murmur at lower left sternal border
The typical flow of blood across an atrial septal defect occurs in which of the following fashions?
A. Deoxygenated blood from left to right atria
B. Deoxygenated blood from right to left anterior descending coronary arteries
C. Oxygenated blood from left to right atria
D. Oxygenated blood from right to left atria
C. Oxygenated blood from left to right atria
ASD*Wide, fixed split S2
*Most common type: ostium secundum
What genetic abnormality has to be present in patients to receive a diagnosis of CML?
Philadelphia chromosome, 9-22 translocations, bcr-abl1 positive
These are all ways that it could be in a vignette, know them all, I have seen them all
TX: Gleevec (Imatinib)
Mnemonic: Tyrone (tyrosine kinase inhibitor first line tx) is a Country Music Lover and was excited to go to a concert in Philadelphia. It was scheduled on 9/11, unfortunately tragedy struck that day and it was rescheduled to 9/22.
A 31 yo female is diagnosed w/ her sixth spontaneous abortion. She has a significant medical history of Lupus. Which of the following is the most likely underlying diagnosis?
A. Antiphospholipid antibody syndrome
B. Hemophilia A
C. TTP
D. Von Willebrand disease
A. Antiphospholipid antibody syndrome
Tx: Hydroxychloroquine for patients w/ SLE
A 33 yo female is admitted to the ICU after a C-section at 38 weeks gestation. Her postoperative period was complicated by a blood clot that was found in her inferior vena cava. The clot was removed via thrombectomy. After the thrombectomy, the patient was oozing blood from the thrombectomy access site for over an hour. On labs, PT and PTT are both increased, fibrinogen is decreased, and D dimer is increased. What is the most likely diagnosis?
A. TTP
B. DIC
C. ITP
D. Hemophilia A
B. DIC
DDIC
D dimer elevated
Dripping (oozing)
Ill
Clots
A 6-month-old infant presents with peripheral edema and hypertension. Skin color is normal. A murmur is not appreciated. However, the blood pressure is markedly higher in the arms then legs. There is also a delayed femoral pulse. Which of the following is the most likely diagnosis?
A. Coarctation of the aorta
B. Transposition of the great arteries
C. Patent ductus arteriosus
D. Tetralogy of Fallot
A. Coarctation of the aorta
*Highly associated w/ Turner syndrome
CXR: 3 sign and rib notching
You are performing a newborn exam on a one-day-old baby girl. In doing so, you hear a continuous, rough, machinery-like murmur during cardiac auscultation. Which of the following heart lesions is most likely associated with the murmur you heard?
A. Atrial septal defect
B. Patent ductus arteriosus
C. Still’s murmur
D. Ventricular septal defect
B. Patent ductus arteriosus
*The ductus arteriosus (connection between pulmonary artery and aorta) in utero shunts blood to the aorta, bypassing fetal lungs, and normally closes within the first five days of life
-When DA remains open longer than one week, it is referred to as PDA
A 62-year-old female presents to the emergency room with complaints of severe, whole-body itching. She states that she first noticed her symptoms while in the bathtub at home. She has never had symptoms like this before. However, over the previous several months she has had episodes of severe joint swelling and pain in her hands as well as redness, burning pain, and swelling of her hands and feet.
Labs reveal elevated RBC count, hemoglobin, and hematocrit of 54%, leukocytosis with a WBC count of 19,000 cells/mm^3 with a normal differential, and thrombocytosis with a platelet count of 900,000/mm^3.
For the most likely diagnosis, what is first line treatment?
Dx: Polycythemia Vera
Tx: repeated phlebotomy to lower hematocrit to ≤ 42%
A 5yo female is brought to the clinic for evaluation of petechiae. The parents deny fever, decreased appetite, bone or joint pain, or weight loss. She did have a cold and runny nose about one month ago. Vitals signs are WNL. PE shows petechiae and purpura on the upper and lower extremities. No signs of mucosal bleeding. Normal lymph nodes, liver, and spleen. Lab studies reveal platelet count of 60,000/microL (low) and an otherwise normal CBC. Which of the following is the most likely diagnosis?
A. HUS
B. ITP
C. Leukemia
D. TTP
B. ITP
*ITP = isolated thrombocytopenia, normal smear
*TTP has CNS symptoms and has schistocytes
ITP Tx: Steroids, If severe → IVIg
A previously healthy 20 yo male presents to the ED assisted by his roommates. The patient is febrile, disoriented, has difficulty speaking, and is complaining of generalized abdominal pain. He has a rash that does not blanch on palpation. Lab findings include platelets 10,000/mcL (low) and creatinine 4.75 mg/dL (high). Coag panel is normal, and peripheral smear shows schistocytes. Which of the following is the most appropriate next step in management?
A. Begin emergent plasma exchange
B. Hold treatment until labs for ADAMTS13 deficiency are completed
C. Prescribe cortisone cream and have the patient follow up with their primary care
D. Watchful waiting as the condition is self-limiting
A. Begin emergent plasma exchange (plasmapheresis)
*Dx: TTP: ADAMTS13 cleaves VWF, TTP is ADAMTS13 deficiency → VWF not regulated → clotting
TTP= FAT RN
Fever
Anemia (MAHA) – schistocytes
Thrombocytopenia – severe < 30 → mucocutaneous bleeding, petechiae, purpura
Renal failure – increased creatinine
Neuro changes – HA, confusion, stroke, seizure
A 4-hour-old male newborn has perioral discoloration for the past several minutes. Oxygen by nasal cannula does not improve the cyanosis. The mother has type 2 diabetes mellitus that was well-controlled during the pregnancy. She has not received any immunizations since her childhood. The newborn's temperature is 37.1°C (98.8°F), pulse is 170/min, respirations are 55/min, and blood pressure is 80/60 mm Hg. Pulse oximetry shows an oxygen saturation of 85%. Cardiopulmonary examination shows a 2/6 holosystolic murmur along the lower left sternal border. The abdomen is soft and non-tender. Echocardiography shows pulmonary arteries arising from the posterior left ventricle, and the aorta rising anteriorly from the right ventricle. Which of the following is the most appropriate next step in management?
A. Reassurance and close observation
B. Prostaglandin E1 administration
C. Indomethacin administration
D. Surgical repair
E. CT angiography
B. Prostaglandin E1 administration
*Dx: transposition of the great arteries
*Must maintain PDA in order for pulmonary and systemic circulation to mix
*Surgical repair (arterial switch operation) is required in all neonates with TOGA but is delayed for 2-3 days until high pulmonary vascular resistance of fetal circulation decreases
A 67-year-old male with a history of recent fracture that occurred incidentally while he was walking reports fatigue and back pain, along with general achiness and "pain in his bones." While reviewing his chart you note he has been treated for multiple infections recently. Recent lab work demonstrated hypercalcemia, increased β2 microglobulin, an increased BUN and Cr as well as a peripheral smear that demonstrated an abnormal stacking of RBCs (rouleaux formation). Serum protein electrophoresis reveals a M-Protein, Urine protein electrophoresis demonstrates Ig light chains (Bence Jones protein). There are punched out lesions on x-ray. What is the most likely diagnosis?
Dx: Multiple Myeloma