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Flashcards in Heme Deck (53):
1

How to work up microcytic anemia?

Ferritin, fe, %sat, TIBC

2

How to work up normocytic anemia?

T.bili/direct bili
LDH
Haptoglobin

3

How to work up macrocytic anemia?

Megaloblastic vs nonmegaloblastic
If megaloblastic, get b12 folate mma.

4

Causes of nonmegaloblastic macrocytic anemia?

Liver disease, etoh, drugs (haart, 5fu), metabolic (lesch-nyhan, orotic aciduria).

5

Neurologic side effects of B12 deficiency?

DCML destroyed -- loss of proprioception. Irreversible.

6

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)

7

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.

8

How to treat thalassemia minor and thalassemia major?

Minor- no treatment
Major -- transfuse, be careful about iron overload. Give desferoxamine.

9

Causes of sideroblastic anemia

B6 deficiency, cancer, MDS -- reversible
Reversible forms: EtoH, lead, low copper.

10

Sickle Cell anemia diagnosis?

Do a hemoglobin electrophoresis. In a crisis? Look at smear for sickling.

11

How to treat sickle cell crisis?

IVF, O2, pain control.

If priapism, acute chest, acute brain? Exchange transfusion

12

G6PD Deficiency

Oxidative stress caused by drugs, fava beans, etc causes rbc lysis.

13

Cells in G6PD

Heinz bodies leading to bite cells

14

How to diagnose G6PD deficiency?

Look at G6PD levels 6-8 weeks after event. Can't check it during hemolysis.

15

Hereditary spherocytosis

Defect in ankyrin or spectryn, loss of central pallor. Hemolyzed in spleen.

16

How to treat hereditary spherocytosis

IVIG, splenectomy, folate, iron

17

How to treat warm hemolysis?

Steroids or eculizumab

18

PNH

Lack DAF, complement fixation during the night leads to intravascular hemolysis. Cells are CD55 negative. Platelets

19

Presenting signs of chronic leukemia

Increased white count, smear normal. Patient usually asymptomatic

20

Presenting signs of acute leukemia

Anemia, bleeding, fevers, bone pain. Younger patients.

21

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

22

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

23

CML

Patient is middle aged, CBC shows neutrophils. 9,22 translocation treat with gleevec.

Can go into blast crisis which is a killer

24

CLL

Seen in old men, chronic
Treatment is for men

25

Lymphoma presenting symptoms

Nontender lymphadenopathy +/- B symptoms (fever, weight loss, night sweats)

26

How to work up possible lymphoma?

Excisional biopsy

27

4 possible results of excisional ln biopsy

Another cancer = met
Nothing = fungus, bacteria, TB
+RS cells = Hodgkins
-RS cells = NHL

28

Next step after diagnosing lymphoma?

Stage by CXR, CT abd and pelvis or pet ct, bone marrow biopsy

29

How to treat based on stage

Stage 2a or better, radiation
Stage 2b or worse, chemo

30

How to stage

Stage 1 = 1 LN
IIA = 2+ LN on same side of diaphragm
III= 2+ on both sides of diaphragm
IV = mets

31

Hodgkins lymphoma

+ b symptoms
+ RS cells
Contiguous spreading
Pel-Epstein: fevers that come and go
Alcohol makes LN painful

32

How to treat hodgkins lymphoma

ABVD or BEACOPP

33

Nonhodgkins lymphoma

- B symptoms
Usually diagnosed at 2b or worse
Hematogenous spreading
Burkitt's -> starry sky.
Extranodal disease

34

How to treat nonhodgkins

Tx with CHOPPER w/ CNS Ppx

35

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

36

Secondary hemostasis

XII, XI, IX, VIII, X, thrombinogen -Thrombin, fibrinogen fibrin

37

Factor trouble leads to

Deep bleeding, prolonged bleeding after surgery. Hemarthroses

38

Platelet dysfunction leads to

Petechiae, gums, vaginal bleeding

39

Von Willibrand Disease

Defect in VWF so problem with ptt, bleeding time
Because of factor 8 insufficiency too.

Tx with DDAVP.

40

Bernard soulier
Glansmann's

Defect in GP1B
Gp2b3a

41

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

42

Time course for HIT if not heparin naive?

1-2 days. much faster than 5-7 days

43

TTP

Defect in ADAMSTS13 which cleaves VWF multimers. Decrease in platelets. Causes renal failure, AMS, hemolytic anemia, fever.

44

Treatment for TTP

Exchange transfusion. NEVER GIVE PLATELETS.

45

ITP

Woman, diagnoses of exclusion, IVIG if acute, long term? Steroids or splenectomy.

46

DIC

You know this

47

Acquired factor disease

Due to liver disease or vitamin D deficiency. Increased PT PTT INR. Give K, if doesn't correct? then liver disease.

48

How to work up clotting diseases?

Factor 5 Leiden mutation, Prothrombin G20210A mutation, Protein C protein S, atIII levels, Lupus anticoagulant

49

Multiple myeloma

Recurrent infections, lytic bone lesions, renal failure.

Work up with SPEP, UPEP, Skeletal survey.

Confirm with BMBX >10% plasma cells. Treat if

50

MGUS

+Protein gap but

51

Sxs of multple myeloma

Calcemia
Renal failure
Anemia
Bone pain

52

Waldenstroms

IGM secretion, hyperviscosity w/stroke, MI.

SPEP is positive
UPEP is negative
SS is negative
BMBX is >10% lymphocytes

53

Waldenstrom's Bone Marrow Biopsy

BMBX>10%