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Step Nuggets > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Surface receptor for macrophage

A

CD14 - this is the LPS receptor

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2
Q

Causes of eosinophilia

A

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

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3
Q

If you see Basophilia

A

CML

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4
Q

AB blood group

A

Universal blood receiver, universal donor of plasma

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5
Q

O blood group

A

Universal donor of blood, universal receiver of plasma

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6
Q

Antithrombin inactivates…

A

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

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7
Q

Test for VWF disease

A

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

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8
Q

Bernard-Soullier disease

A

Defect in GpIb

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9
Q

Glanzmann Thrombasthenia

A

Defect in GpIIb/IIIa- works like abciximab

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10
Q

Reasons for Basophillic stippling

A

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

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11
Q

Reasons for Target cells

A

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

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12
Q

Plummer Vinison syndrome

A

Iron deficiency anemia, esophageal webs, atrophic

glossitis

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13
Q

Hb Barts

A

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

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14
Q

HbH

A

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

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15
Q

HbF

A

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

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16
Q

HbA2

A

alpha2delta2

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17
Q

Cause of beta thalassemia

A

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

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18
Q

Lead poisoning

A

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.

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19
Q

Causes of sideroblastic anemia

A

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

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20
Q

Orotic aciduria

A

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

21
Q

Signs of intravascular hemolysis

A

Increased LDH, decreased haptoglobin, increased hemoglobinuria

22
Q

Signs of extravascular hemolysis

A

Increase in unconjugated hyperbilirubinemia (Jaundice). Increased LDH

23
Q

Hereditary spherocytosis

A

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

24
Q

Paroxysmal Nocturnal Hemoglobinuria

A

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
Q

Auto Immune Hemolytic Anemia with warm agglutinin

A

IgG Ab. Seen in SLE, CLL, Methyldopa treatment

26
Q

Cold Agglutinin

A

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

27
Q

Acute intermittent porphyria

A

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
Q

Porphyria Cutanea Tarda

A

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

29
Q

Hemarthrosis, easy bruising points to…

A

Coagulation disorder

30
Q

Mucosal bleeding, epistaxis, petichiae points to…

A

Platelet disorder

31
Q

Autoimmune thrombocytopenia

A

Ab against GpIIb/IIIa

32
Q

TTP

A

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
Q

Treatment for VWf disease

A

DDAVP (increased VWf release)

34
Q

Can ATIII deficiency be cause by nephritic syndome?

A

No. But it can with a nephrotic syndrome.

35
Q

Blood transfusions can lead to…

A

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

36
Q

Difference between leukemoid reaction and CML

A

Leukemoid reaction has high Leukocyte ALP, low in CML.

37
Q

50% of Hodgkin lymphoma are associated with this virus

A

EBV

38
Q

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

A

Ig heavy chain (Highly active in B cells)

39
Q

Mantle Cell lymphoma

A

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

40
Q

Follicular lymphoma

A

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

41
Q

Pseudo-Pelger-Huet anomaly

A

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

42
Q

Leukemia in someone <15

A

ALL (Goljan)

43
Q

Leukemia in middle aged person

A

AML or CML (Goljan)

44
Q

Leukemia in old person

A

SLL/CLL (Goljan)

45
Q

Hairy cell leukemia

A

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

46
Q

Langerhans Cell histocytosis

A

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
Q

JAK2 mutation

A

Myeloproliferative disorder

non-receptor tyrosine kinase

48
Q

Erythromelalgia

A

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

Step Nuggets (23 decks)

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