JH IM Board Review - SOS V Flashcards
The ristocetin test is abnormal in which patients:
- VWD.
2. B-S syndrome.
Which specific lab tests are important to be ordered since they may all manifest as thrombocytopenia?
- Liver disease.
- HCV.
- HIV.
- Pregnancy.
4 inherited platelet disorders:
- Glanzmann.
- B-S.
- Gray platelet syndrome.
- Storage pool disease.
Gray platelet syndrome — Defect:
Deficiency of alpha granules.
Storage pool disease — Defect:
Deficiency of dense granules.
Pts w/ gray platelet syndrome or storage pool disease often have only …
MILD bleeding manifestations.
Infections that may lead to thrombocytopenia:
- HIV.
- CMV.
- EBV.
- HANTAVIRUS.
- Viral hep.
- Mycoplasma.
Clinical presentation of ACQUIRED platelet disorders typically results in bleeding.
Mention the 2 exceptions:
- HIT type 2 ==> a/w thrombosis.
2. Myeloproliferative disorders ==> May have bleeding or thrombosis.
Medical disease-related platelet disorders (specifically LIVER disease) may result in apparent thrombocytopenia caused by sequestration but …
NO clinical bleeding disorder.
HIT type 1:
NON immune-mediated.
Begins a few days after initiation of heparin + will spontaneously resolve if heparin is CONTINUED.
HIT type 2:
Immune-mediated development of abs against heparin/PAF-4 complex.
Begins 5-10 days after initiation of heparin.
Heparin must be DISCONTINUED immediately in which HIT?
2
What should be administered in HIT 2 after the discontinuation of the heparin?
DIRECT THROMBIN INHIBITOR.
HIT 2 or HIT 1 is a/w thromboses?
HIT 2 ==> Can present 40 days (!) post-heparin exposure.
HIV and HCV cause thrombocytopenia directly or indirectly?
DIRECTLY.
Thrombosis in pts w/ thrombocytosis is NOT predicted by the platelet count.
Which 2 factors are the best predictors?
- Advanced age.
2. Previous thrombosis.
Palpable vs non palpable purpura?
Palpable ==> Vessel wall inflammation (eg vasculitis).
Non-palpable ==> Vessel wall disorders or connective tissue disorders.
Protein S levels are reduced by E…
ESTROGENS.
If homocysteine testing is performed, blood samples should be drawn after f…
FASTING.
Lab monitoring of LMWH is usually NOT necessary.
If monitoring is warranted, use the L…
LMW (anti-Xa) assay.
LMWH are or aren’t reversible with protamine?
60-80% they are partially reversible.
LMWH should be used w/ caution in pts w/ R…
Renal insufficiency. (GFR <30)
INR limits:
2-3 ==> VTE — Aortic bileaflet mechanical heart valves — A-fib.
2.5-3.5 ==> Mitral bileaflet mechanical heart valves — Recurrent VTE — APS w/ recurrent thromboembolism.
5 syndromes that increase AML risk:
- Down.
- Bloom.
- Fanconi.
- ATM.
- Klinefelter (!).
In AML, leptomeningeal involvement is more common with e… at diagnosis or M…
Elevated WBC count or M4/M5 morphology.
S… is uncommon in AML.
Splenomegaly.
Low WBC in AML raises suspicion of which subtype?
M3/APL.
Clinical features a/w poor prognosis in AML include:
- > 60y.
- Poor performance status.
- Therapy-related AML (2o to chemo).
- AML arising from another marrow disorder (MDS or myeloproliferative).
- WBC >100K (hyperleukocytosis).
AML prognosis — Expected …-…% of pts >60y and
35-40%.
10%.
Up to …% of ALL pts will present w/ fever caused by either pyrogenic cytokine release or true concurrent infection.
50%.
Evaluation of ALL always includes analysis of C…
CSF for CNS involvement.
In CML — anemia, thrombocytopenia, and monocytosis are red flags that you may …
NOT be dealing w/ CHRONIC-phase CML.
Tx-related or chemo-related MDS — Topoisomerase II inhibitors (eg anthracyclines, etoposide):
A/w which cytogenetic abnormality?
11q23.
Typical onset between 1 and 3 years after chemotherapy.
Tx-related or chemo-related MDS — Alkylating agents (eg cyclophosphamide):
A/w unbalanced cytogenetic changes including abnormalities of chromosome 5 or 7.
Typical onset is 5-7y post-chemo. (Hah same chromosomes and same year)
In MDS, the cytogenetic abnormality predicts the o…
Outcome.
Early MDS:
Increased levels of apoptosis + increased proliferation ==> Hypercellular BM w/ peripheral cytopenias.
Later MDS:
Apoptosis decreases and proliferation increases ==> More aggressive disease and possible transformation to AML.
MDS — Order of cells affected?
RBCs ==> WBCs ==> PLTs.
Target cells — DDx:
- Hemoglobin C.
- Liver disease.
- Thalassemia.
Basophilic stippling — DDx:
- Hemolysis.
- Lead.
- Thal.
Burr cells — DDx:
Renal disease.
Acanthocytes — DDx:
- Liver disease.
2. Abetalipoproteinemia.
Leukopenia w/ HYPOsegmented PMNs — DDx:
- MDS.
- Stress.
- Infection.
- Pelger-Huet anomaly.
Indications for a BM biopsy and aspirate:
- Unexplained cytopenias — Young pts w/ ITP and no other hematologic abnormalities or dysplasia may not need a BM evaluation.
- Suspected hematologic malignancies (leukemia, MDS, lymphoma, plasma cell dyscrasia).
- FUO.
- Storage disease.
- Splenomegaly.
- Chromosomal abnl in neonates.
- Donor selection process for SCT.
CI to BM biopsy and aspirate:
- Coagulation defects — should be corrected before procedure.
Eg hemophilia, DIC, heparin, warfarin, direct thrombin inhibitors.
- Local infections at the site.
*** Thrombocytopenia (regardless of number) is NOT a CI for BM biopsy.
Lynch — Extracolonic cancer w/ highest incidence?
Endometrial.
CRC w/ pelvic pain usually indicates L…
Late-stage disease — extension into pelvic nerves.
Adjuvant chemo in colorectal cancer has been shown to s…
Significantly improve survival in pts w/ STAGE III disease.
The 5 polyposis syndromes:
- FAP.
- Gardner.
- Turcot.
- Peutz-Jeghers.
- Juvenile polyposis.
CRC recurrence evaluation — If elevated CEA is found, then c…
Colonoscopy + CT scans (pelvis, abdo, chest) should be performed.
If negative ==> REPEAT every 3mo.
If positive ==> Salvage therapy.
Mention 3 unfavorable prognostic factors of CRC:
- Long duration of symptoms.
- Bowel obstruction or perforation.
- Elevated PRE-op CEA.
The BRCA genes are not X-linked, but are s… in most men.
SILENT.
DCIS — Tamoxifen role?
Decreases the risk of a contralateral cancer and decreases IN-BREAST recurrence after lumpectomy and RT, but it does NOT improve survival.
Breast cancer — Choosing between lumpectomy + RT and mastectomy — Survival is e…
Equivalent.
…% of pts w/ advanced ovarian cancer have an elevated CA-125, but
82%.
50%.
Autopsy studies suggest that nearly …% of men will have prostate cancer by age 80.
60%.
Prostate cancer extension to seminal vesicles may manifest w/ h…
Hematospermia.
PSA may be elevated from:
- BPH.
- Acute prostatitis.
- Transrectal needle biopsy.
- Acute urinary retention.
- Prostate surgery.
- Ejaculation.
Side effects of androgen ablation:
- Hot flashes.
- Sexual dysfunction (loss of libido, erectile dysfunction, microgenitalia).
- Decreased bone and muscle mass.
- Gynecomastia.
- Fatigue — Anemia.
- Metabolic syndrome.
- Neurocognitive effects.
What is Balkan nephropathy?
A familial nephropathy of unknown cause that results in progressive inflammation of the renal parenchyma ==>
Renal failure + Multifocal, superficial, low-grade cancers of the renal pelvis and ureters.
What is Stauffer syndrome?
Hepatic dysfunction NOT a/w metastases in RCC pts.
In RCC, does renal vein involvement affect survival?
No.
Sunitinib may cause …
Decreased cardiac EF.
False(+) elevations can be seen with h… and the use of m… .
Hypogonadism and the use of marijuana.
Functional incontinence occurs when …
An individual is unable or unwilling to reach the toilet in time.
Functional incontinence is a/w …
- Dementia/depression.
- Physical impairments that impede motility (eg degenerative arthritis, stroke, PD).
- Physical restraints or inaccessible toilets.
Mention the 4 stages of pressure ulcers:
Stage 1 ==> Nonblanchable erythema.
Stage 2 ==> Irregular shallow ulceration — Loss of epidermis, dermis, or both.
Stage 3 ==> Deep ulceration with necrotic base.
Stage 4 ==> Deep ulceration reaching underlying bone.
Pressure ulcers and infections:
Colonization of the ulcer is common and does not represent infection.
Swab cultures of the wound base have NO clinical utility.
DO NOT use topical antibiotics.
Falls — Where do they happen?
70% ==> At home.
10% ==> On the stairs.
50% ==> Environmental hazards (Eg wet surface, throw rugs).
Which meds are a/w the greatest risk for falling?
- Sedatives.
- Antidepressants.
- Neuroleptics.
Cataract — Morphological types:
- Nuclear sclerosis.
- Posterior subcapsular.
- Cortical.
Most cataracts are age-related.
Mention some causes of cataract:
- Steroids.
- Trauma.
- DM.
- FHx.
- Uveitis.
- Radiation.
- Wilson — SUNFLOWER cataract.
- Prior ocular surgery — especially pars plana vitrectomy.
Does cataract have pain/redness?
NO — EXCEPT in cases of lens-induced glaucoma or uveitis.
Risk factors for progression of “wet”, neovascular age-related macular degeneration?
- HTN.
2. Smoking.
Clinical presentation of age-related macular degeneration:
- Distortion of STRAIGHT LINES may be an early sign.
- No pain/no redness.
- Peripheral vision remains INTACT.
Prevention of age-related macular degeneration:
- Sunglasses or hats to block UV light.
- Control of BP to reduce risk of wet AMD may also be helpful.
- Cessation of smoking strongly encouraged.
Primary open-angle glaucoma screening in asx pts:
- Recommended every 2-4y for 40-64yo pts.
- Every 1-2y for >65y.
- Begin before 40y in African Americans.
Aminoglycosides may lead to what eye problem?
Toxic or allergic keratoconjunctivitis.
What should be strictly avoided in HSV keratitis?
CS
MC ocular manifestation of HIV:
Background HIV retinopathy (non infectious).
Immune recovery uveitis (IRU) occurs in minority of eyes w/ CMV retinitis in pts responsive to HAART:
Syndrome of uveitis + vitreitis + macular edema + epiretinal membrane formation.
RFs for IRU unclear.
