Heme/Onc Flashcards Preview

Step Nuggets > Heme/Onc > Flashcards

Flashcards in Heme/Onc Deck (48):
1

Surface receptor for macrophage

CD14 - this is the LPS receptor

2

Causes of eosinophilia

NAACP- Neoplasia, Asthma, Allergies, Connective tissue disease, Parasites

3

If you see Basophilia

CML

4

AB blood group

Universal blood receiver, universal donor of plasma

5

O blood group

Universal donor of blood, universal receiver of plasma

6

Antithrombin inactivates...

2, 7, 9, 10, 11, 12. Main function is to inactivate IIa and Xa

7

Test for VWF disease

decreased agglutination on Ristocetin assay, ristocetin activates VWf to bind to Gp1b

8

Bernard-Soullier disease

Defect in GpIb

9

Glanzmann Thrombasthenia

Defect in GpIIb/IIIa- works like abciximab

10

Reasons for Basophillic stippling

LATA (Latte)- Lead poisoning, Alcohol, Thalassemia, Anemia of chronic disease

11

Reasons for Target cells

HALT- Hemoglobin C disease, Asplenia, Liver disease, Thalassemia

12

Plummer Vinison syndrome

Iron deficiency anemia, esophageal webs, atrophic
glossitis

13

Hb Barts

No a globin, leads to hydrops fetalis. Hb Barts is a gamma4 formation. (Common in asia)

14

HbH

Three alpha globin gene deletion. Leads to beta4 conformation, called hemoglobin H.

15

HbF

alpha2gamma2 (Think of a baby in a crib, the gamma sign looks like a crib? Maybe?)

16

HbA2

alpha2delta2

17

Cause of beta thalassemia

point mutations in splice sites and promoters, defect in alternative splicing.

18

Lead poisoning

Inhibits ferrochelatase and ALA dehydratase. LEAD- Lead lines on gingivae and long bones of x-ray (burton lines), Encephalopathy and erythrocyte basophilic stippling, Abdominal colic or Sideroblastic Anemia, Wrist and foot Drop, Dimercaprol, EDTA, Succimer used for chelation.

19

Causes of sideroblastic anemia

X-Linked defect in DALA sythase (RLS of Heme synthesis). Lead poisoning. Alcohol (MOST COMMON). Pyridoxine deficiency.

20

Orotic aciduria

Defect in pyrimidine synthesis (UMP synthesis). WILL NOT SEE HYPERAMMONEMIA like you will in OTC deficiency. Give Uridine Monophosphate.

21

Signs of intravascular hemolysis

Increased LDH, decreased haptoglobin, increased hemoglobinuria

22

Signs of extravascular hemolysis

Increase in unconjugated hyperbilirubinemia (Jaundice). Increased LDH

23

Hereditary spherocytosis

Osmotic fragility test +. Can do Eosin-5-Maleimide test for screening. Increased MCHC, extravascular hemolysis.

24

Paroxysmal Nocturnal Hemoglobinuria

No GPI anchor to decay accelerating factor to stop complement from lysis. CD55/59 negative. Coombs negative hemolysis, pancytopenia, venous thrombosis. treat with Eculizumab

25

Auto Immune Hemolytic Anemia with warm agglutinin

IgG Ab. Seen in SLE, CLL, Methyldopa treatment

26

Cold Agglutinin

IgM. Seen in Mononucleosis and Mycoplasma Pneumo (All M's)

27

Acute intermittent porphyria

No porphobilinogen deaminase. Acumulation of porphobilinogen, DALA, coporphobilinogen (urine). Symptoms : 5P’S- PAINFUL ABDOMEN, PORT WINE COLORED URINE, POLYNEUROPATHY, PSYCH DISTURBANCES, PRECIPITATION BY DRUGS, ALC, STARVATION Treat with glucose and heme, inhibiting ALA synthase

28

Porphyria Cutanea Tarda

Defect in Uroporphyrinogen Decarboxylase. Tea colored urine, blistering cutaneous photosensitivity.

29

Hemarthrosis, easy bruising points to...

Coagulation disorder

30

Mucosal bleeding, epistaxis, petichiae points to...

Platelet disorder

31

Autoimmune thrombocytopenia

Ab against GpIIb/IIIa

32

TTP

Defect in ADAMTS13. Large VWf multimers lead to easy clotting in microvasculature but doesn't use up coag factors so you're not getting DIC. Neuro and renal symptoms, thrombocytopenia, microangiopathic anemia. Treat with exchange transfusion and steroids.

33

Treatment for VWf disease

DDAVP (increased VWf release)

34

Can ATIII deficiency be cause by nephritic syndome?

No. But it can with a nephrotic syndrome.

35

Blood transfusions can lead to...

Hyperkalemia (due to blood cell lysis in storage), Hypocalcemia (blood is stored with citrate, a Ca2+ chelator), Iron overload.

36

Difference between leukemoid reaction and CML

Leukemoid reaction has high Leukocyte ALP, low in CML.

37

50% of Hodgkin lymphoma are associated with this virus

EBV

38

What gene is on chromosome 14 that creates all these lymphomas when a different gene is translocated to its spot?

Ig heavy chain (Highly active in B cells)

39

Mantle Cell lymphoma

CD5+. 11:14 translocation. Cyclin-D overexpressed.

40

Follicular lymphoma

14:18 translocation. BCL2 overexpressed (prevents apoptosis through mitochondrial stabilization). Can see waxing and waning lymphoma.

41

Pseudo-Pelger-Huet anomaly

Bilobed PMN with single strand of chromatin connecting the lobes. Seen in chemo treatment

42

Leukemia in someone <15

ALL (Goljan)

43

Leukemia in middle aged person

AML or CML (Goljan)

44

Leukemia in old person

SLL/CLL (Goljan)

45

Hairy cell leukemia

TRAP+. Dry tap bone marrow. Treat with cladribine which inhibits Adenosine deaminase.

46

Langerhans Cell histocytosis

Presents in CHILD as rash, lytic bone lesions, recurrent otitis media with MASS OF MASTOID BONE. CD1a+, S100+ (mesodermal origin). Tennis racket birbeck granules.

47

JAK2 mutation

Myeloproliferative disorder
non-receptor tyrosine kinase

48

Erythromelalgia

Polycythemia vera sort of pain crisis- Severe burning pain with reddish blue color of skin due to clots in extremity vasculature.