Heme Flashcards
How to work up microcytic anemia?
Ferritin, fe, %sat, TIBC
How to work up normocytic anemia?
T.bili/direct bili
LDH
Haptoglobin
How to work up macrocytic anemia?
Megaloblastic vs nonmegaloblastic
If megaloblastic, get b12 folate mma.
Causes of nonmegaloblastic macrocytic anemia?
Liver disease, etoh, drugs (haart, 5fu), metabolic (lesch-nyhan, orotic aciduria).
Neurologic side effects of B12 deficiency?
DCML destroyed – loss of proprioception. Irreversible.
Thalassemia
Problem in globin creation.
Alpha thalassemia (1 missing=asx, 2 missing = mild, 3 missing = severe, 4 missing = hydrops fetalis)
Beta-thalassemia (1 missing mild, 2 missing severe)
How to diagnose thalassemia? How to distinguish between alpha and beta thal?
Hemoglobin electrophoresis. Beta thalassemia has weird hemoglobins (HbA2, HbF). Alpha thalassemia is a diagnosis of exclusion.
How to treat thalassemia minor and thalassemia major?
Minor- no treatment
Major – transfuse, be careful about iron overload. Give desferoxamine.
Causes of sideroblastic anemia
B6 deficiency, cancer, MDS – reversible
Reversible forms: EtoH, lead, low copper.
Sickle Cell anemia diagnosis?
Do a hemoglobin electrophoresis. In a crisis? Look at smear for sickling.
How to treat sickle cell crisis?
IVF, O2, pain control.
If priapism, acute chest, acute brain? Exchange transfusion
G6PD Deficiency
Oxidative stress caused by drugs, fava beans, etc causes rbc lysis.
Cells in G6PD
Heinz bodies leading to bite cells
How to diagnose G6PD deficiency?
Look at G6PD levels 6-8 weeks after event. Can’t check it during hemolysis.
Hereditary spherocytosis
Defect in ankyrin or spectryn, loss of central pallor. Hemolyzed in spleen.
How to treat hereditary spherocytosis
IVIG, splenectomy, folate, iron
How to treat warm hemolysis?
Steroids or eculizumab
PNH
Lack DAF, complement fixation during the night leads to intravascular hemolysis. Cells are CD55 negative. Platelets
Presenting signs of chronic leukemia
Increased white count, smear normal. Patient usually asymptomatic
Presenting signs of acute leukemia
Anemia, bleeding, fevers, bone pain. Younger patients.
AML
Seen in patients in their late 20’s usually. Can go into DIC, have auer rods. Show myeloperoxidate. Treat M3 with ATRA. Otherwise with chemo
ALL
Seen in young kids and downs syndrome.
Bone marrow biopsy reveals >20% blasts stain with tdt. Treat with chemo, make sure to give prophylaxis with AraC
CML
Patient is middle aged, CBC shows neutrophils. 9,22 translocation treat with gleevec.
Can go into blast crisis which is a killer
CLL
Seen in old men, chronic
Treatment is for men
Lymphoma presenting symptoms
Nontender lymphadenopathy +/- B symptoms (fever, weight loss, night sweats)
How to work up possible lymphoma?
Excisional biopsy
4 possible results of excisional ln biopsy
Another cancer = met
Nothing = fungus, bacteria, TB
+RS cells = Hodgkins
-RS cells = NHL
Next step after diagnosing lymphoma?
Stage by CXR, CT abd and pelvis or pet ct, bone marrow biopsy
How to treat based on stage
Stage 2a or better, radiation
Stage 2b or worse, chemo
How to stage
Stage 1 = 1 LN
IIA = 2+ LN on same side of diaphragm
III= 2+ on both sides of diaphragm
IV = mets
Hodgkins lymphoma
\+ b symptoms \+ RS cells Contiguous spreading Pel-Epstein: fevers that come and go Alcohol makes LN painful
How to treat hodgkins lymphoma
ABVD or BEACOPP
Nonhodgkins lymphoma
- B symptoms Usually diagnosed at 2b or worse Hematogenous spreading Burkitt's -> starry sky. Extranodal disease
How to treat nonhodgkins
Tx with CHOPPER w/ CNS Ppx
Primary hemostasis
Tissue factor exposed, binds vWF, then binds GP1B, then ADP receptor binds ADP, then Gp2b/3a binds fibrinogen, gets cross linked to fibrin
Secondary hemostasis
XII, XI, IX, VIII, X, thrombinogen -Thrombin, fibrinogen fibrin
Factor trouble leads to
Deep bleeding, prolonged bleeding after surgery. Hemarthroses
Platelet dysfunction leads to
Petechiae, gums, vaginal bleeding
Von Willibrand Disease
Defect in VWF so problem with ptt, bleeding time
Because of factor 8 insufficiency too.
Tx with DDAVP.
Bernard soulier
Glansmann’s
Defect in GP1B
Gp2b3a
HIT
5-7 days post heparin platelets drop by 50% due to heparin platelet factor 4 immune complex formation.
Confirm with HIT antibody.
Tx by switching to argatroban
Time course for HIT if not heparin naive?
1-2 days. much faster than 5-7 days
TTP
Defect in ADAMSTS13 which cleaves VWF multimers. Decrease in platelets. Causes renal failure, AMS, hemolytic anemia, fever.
Treatment for TTP
Exchange transfusion. NEVER GIVE PLATELETS.
ITP
Woman, diagnoses of exclusion, IVIG if acute, long term? Steroids or splenectomy.
DIC
You know this
Acquired factor disease
Due to liver disease or vitamin D deficiency. Increased PT PTT INR. Give K, if doesn’t correct? then liver disease.
How to work up clotting diseases?
Factor 5 Leiden mutation, Prothrombin G20210A mutation, Protein C protein S, atIII levels, Lupus anticoagulant
Multiple myeloma
Recurrent infections, lytic bone lesions, renal failure.
Work up with SPEP, UPEP, Skeletal survey.
Confirm with BMBX >10% plasma cells. Treat if
MGUS
+Protein gap but
Sxs of multple myeloma
Calcemia
Renal failure
Anemia
Bone pain
Waldenstroms
IGM secretion, hyperviscosity w/stroke, MI.
SPEP is positive
UPEP is negative
SS is negative
BMBX is >10% lymphocytes
Waldenstrom’s Bone Marrow Biopsy
BMBX>10%
