Heme/ Onc

Question 1

A child presents with petechiae and low platelets a few weeks after a URI. How would you treat?

Answer 1

observation; if the child is not bleeding no IVIG or steroids are needed because ITP is self-limited in children

Question 2

what are complications of multiple myeloma

Answer 2

renal failure, hypercalcemia, hyperviscosity syndrome

Question 3

what are the symptoms of fanconi anemia and how is diagnosis confirmed

Answer 3

sx=aplastic anemia, progressive bone marrow failure, short stature, microcephaly, curved thumbs, hypogonadism, skin pigmentation abnormalities, low-set ears, middle ear hemorrhages/infections/deafness, horseshoe kidney; dx confirmed via chromosomal breaks on genetic analysis

Question 4

what is the most common complication of sickle cell trait

Answer 4

painless hematuria

Question 5

what is Diamond-Blackfan anemia

Answer 5

a pure red blood cell aplasia that presents at around 3-12 months, macrocytic anemia, WBC and platelet counts are normal
associated congenital abnormalities include webbed neck, short stature, crested chest and triphalyngeal thumbs

Question 6

if a patient becomes pancytopenic after drug, toxin or viral exposure you should suspsect what

Answer 6

acquired aplastic anemia

Question 7

various sites of venous thrombosis in the arms and chest should suggest what condition and what associated disease

Answer 7

Trousseau’s syndrome= hypercoagulable disorder presenting with superficial thrombophlebitis in the chest and upper extremities
Trousseau’s syndrome is associated with occult visceral malignancies

Question 8

at what size would you want to consider biopsy of a lymph node palpated on physical exam

Answer 8

lymph node greater than 2cm (or firm/immobile)

Question 9

describe Wiskott-Aldrich syndrome (inheritance pattern and triad of symptoms)

Answer 9

Wiskott-Aldrich is an X-linked disorder characterized by eczema, thrombocytopenia and hypogammaglobulinemia (leading to recurrent bacterial infections)

Question 10

what is hyposthenuria

Answer 10

inability to concentrate the urine; seen in sickle cell and sickle cell trait; due to sickling of vasa recta of medulla of kidney impairing countercurrent exchange and water reabsorption

Question 11

“soap bubble”-appearing lesion in the epiphysis of a long bone should make you think of what disease

Answer 11

Giant cell tumor

Question 12

what happens to calcium, phosphate, potassium and uric acid in tumor lysis syndrome

Answer 12

Ca= decreased (bound up by excess phosphate), Phosphate=elevated; potassium= elevated; uric acid=elevated

Question 13

what is used to treat DVT caused by homocysteinemia?

Answer 13

vitamin B6 and folate (cofactors for conversion to cysteine)

Question 14

what is the paraprotein gap usually like for patients with multiple myeloma

Answer 14

increased (i.e. difference between total protein and albumin=greater than 3-4g/dL)

Question 15

what are the two main manifestations of paroxysymal nocturnal hemoglobinuria

Answer 15

1. hemolytic anemia

2. vein thromboses (intraabdominal, intracranial)

Question 16

what is the most common cause of megaloblastic anemia in chronic alcoholics

Answer 16

folate deficiency (not B12 deficiency)

Question 17

what is the drug of choice for treating cancer-related cachexia and anorexia

Answer 17

progesterone analogs

Question 18

what are the symptoms of a pinealoma

Answer 18

• Parinaud’s (ptosis, downward gaze, pupil constriction to accomodation only, upper eyelid retraction)
• obstructive hydrocephalus
• precocious puberty for beta-HCG secreting tumors

Question 19

what is first-line therapy for chemo-induced vomiting/nausea and what is its mechanism of action

Answer 19

zofran (odansetron): a serotonin 5HT^3 antagonist

Question 20

after exposure/wound, when should tetanus toxoid vaccine vs. tetanus immunoglobulin

Answer 20

tetanus immunoglobulin should only be given if the wound is severe/dirty AND the patient does not have complete vaccination (>3 toxoid doses), to be given along with toxoid vaccine
just the toxoid vaccine should be given for minor wounds and for patients with incomplete immunization history

Question 21

how do ACE inhibitors predispose to acquired angioedema

Answer 21

at ANY point in the use of ACE inhibitors angioedema can occur due to inhibition of bradykinin breakdown; bradykinin promotes edema

Question 22

should vaccines for premature infants be given according to gestational age or chronological age

Answer 22

chronological age; as long as the premature infant is stable and weight >2kg, vaccines should be given by how old the infant is rather than how long the pregnancy was

Question 23

what is the workup algorithm for a solitary pulmonary nodule

Answer 23

CXR (compare with previous) –> CT scan; if benign do serial CTs to monitor, if indeterminate do biopsy or PET, if highly suspicious for malignancy do surgical excision

Question 24

which pneumococal vaccine produces immunity via T-cell dependent vs. T-cell independent B cell memory

Answer 24

PCV-13 is conjugated and therefore produces a more robust T-cell dependent B cell response whereas PPSV-23 is just polysaccharide and works via T-cell independent mechanism (not as good for kids/elderly)

Question 25

is a seizure after DTaP administration a contraindication to subsequent vaccinations

Answer 25

no; only anaphylaxis, encephalopathy and unstable neurologic disorders

Question 26

what is the cause of hyperIgM syndrome

Answer 26

X-linked defect of CD40 ligand leading to inability to class switch

Question 27

what factors comprise a high risk solitary pulmonary nodule (these must be surgically excised)

Answer 27

size greater than or equal to 2cm
age older than 60
current smoker or quit less than 5 years ago
corona radiata or spiculated

Question 28

what are the side effects of erythropoetin therapy

Answer 28

worsening hypertension, headache, flu-like syndrome, red cell aplasia

Question 29

how can you differentiate between iron deficiency anemia and thalassemia (both are microcytic anemias)

Answer 29

RDW is high in iron deficiency anemia and normal in thalassemias

Question 30

what two things distinguish Waldenstrom’s macroglobulinemia from multiple myeloma

Answer 30

1. IgM spike (vs. IgG or IgA in MM)

2. hyperviscosity (think MACROglobulinemia)

Question 31

in a patient with malignancy who develops sudden onset severe back pain with neuropathies what is the condition and management algorithm

Answer 31

epidural spinal cord compression
severe vs. not-severe: if severe get emergency MRI and give steroids –> radiation or surgery if indicated by MRI
if not severe get an MRI within 24 hours
if no myelopathy just get an x-ray –> MRI if indicated

Question 32

characteristic symptoms of graft vs. host disease

Answer 32

can occur at any time and involves activation of donor T lymphoctyes;
sx= maculopapular rash involving face, hands and soles; bloody diarrhea; jaundice

Question 33

what are the complications of sickle cell trait

Answer 33

gross hematuria due to renal papillary necrosis (which also results from tylenol, diabetes, acute pyelo), UTI’s and renal medullary carcinoma

Question 34

fever, leukocytosis and left upper quadrant pain with associated splenomegaly and pleural effusion is most likely what?
what are the common etiologies of this condition?

Answer 34

splenic abscess; can be due to infective endocarditis, trauma, IVDU, immunosuppression, hemoglobinopathies

Question 35

fever, chills, and deep abdominal pain suggest what

Answer 35

retroperitoneal abscess

Question 36

what are the clinical features of cholesterol emboli

Answer 36

livedo reticularis, blue toe syndrome, gangrene/ulcers, stroke, amaurosis fugax, acute or subacute kidney injury, intestinal ischemia, pancreatitis, Hollenhorst plaques

Question 37

what are the lab findings for cholesterol emboli

Answer 37

elevated creatinine, eosinophilia, hypocomplementemia

eosinophiluria

Question 38

what two diseases should a patient with isolated thrombocytopenia be tested for

Answer 38

HIV and Hep C can present as ITP

Question 39

a child with isolated thrombocytopenia after a viral URI likely has what condition? how do you treat it if it is uncomplicated vs. complicated by active bleeding?

Answer 39

immune thrombocytopenia (ITP)
observation only if uncomplicated (resolves in 6 months)
treat with IVIG or steroids if c/b bleeding
note: adults should get IVIG or steroids for platelets

Question 40

which drugs cause acute pancreatitis

Answer 40

diuretics (furosemide and HCTZ), antibiotics (metronidazole, tetracyclines), drugs for IBD (mesalamine, sulfasalazine), azathioprine, HIV drugs (didanosine, pentamidine)

Question 41

what is the classic triad of disseminated gonococcemia

Answer 41

polyarthralgias, tenosynovitis, painless vesicopustular lesions

Question 42

how do you tell the difference between iron deficiency anemia and thalassemia

Answer 42

RDW: iron deficiency anemia will have a high red cell distribution width due to poikilocytosis as smaller cells are being produced in an iron-deficient state
in thalassemias the RDW is normal