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Step 1 > Heme onc > Flashcards

Heme onc Flashcards

1
Q

Lipid A from bacterial LPS binds ____ on macrophages to initiate septic shock.

A

CD14

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2
Q

What toxins to eosinophils have that fight helminths?

A

Major basic protein

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3
Q

What is mnemonic for fetal erythropoiesis?

A

Young liver synthesizes blood

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4
Q

Review fetal erythropoiesis.

A

Yolk sac (3-8 weeks)
Liver (6 weeks - birth)
Spleen (10-28 weeks)
Bone marrow (18 weeks - adult)

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5
Q

What substrate does bradykinin come from? What enzyme catalyzes this?

A

Bradykinin formed from HMWK via kallikrein

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6
Q

What can activate the intrinsic pw?

A

“Contact activation”

Collagen, BM, activated platelets, HMWK

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7
Q

What can activate the extrinsic pw?

A

Tissue factor

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8
Q

Function of TXA2?

A

(released by platelets)

  • Decreases blood flow
  • Increases platelet aggregation
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9
Q

What are acanthocytes and when do you seen them?

A

RBCs w/spinous-like protrusion

  • Liver disease
  • Abetalipoproteinemias
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10
Q

When do you see basophilic stippling?

A
  • Lead poisoning
  • Sideroblastic anemias
  • Myelodysplastic syndromes
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11
Q

When do you seen teardrop cells (dacrocytes)?

A

Bone marrow infiltration (getting squeezed out)

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12
Q

When do you see bite cells?

A

G6PD def.

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13
Q

What are echinocytes and when do you see them?

A

“Burr cells” (more uniform and smaller processes compared to acanthocytes, which have spinous processes)

  • ESRD
  • Liver disease
  • Pyruvate kinase def.

(Bill Burr)

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14
Q

When can sickling of RBCs occur in SCD?

A
  • Low O2
  • Low H2O
  • Acidosis
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15
Q

When can you see target cells?

A

“HALT,” said the hunter to his target

Hb C disease
Asplenia
Liver disease
Thalassemia

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16
Q

What type of Hb is formed with a 4 gene alpha-thal deletion?

A

Hb Barts (only gamma)

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17
Q

What type of Hb is formed with a 3 gene alpha-thal deletion?

A

Hb B (some Beta tetramers)

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18
Q

S/s of Pb poisoning?

A

LEAD
Lead Lines on gingiva & metaphyses of long bones
Encephalopathy, Erythrocyte basophilic stippling
Abdominal colic and sideroblastic Anemia
Drops (wrist and foot); Dimercaprol & eDta for chelation

Succimer used for chelation in kids (Succ’s to be a kid who eats lead)

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19
Q

What enzymes are inhibited in Pb poisoning? What builds up?

In X-linked sideroblastic anemia?

A

Pb: ALAD, ferrochelatase
- ALA and protoporphyrin buildup

Sideroblastic: ALAS (RLS, requires B6)
- Glycine/succinyl CoA buildup
(this rxn is also inhibited by glucose, heme)

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20
Q

What type of anemia does orotic aciduria cause?

A

Megaloblastic

  • De novo pyrimidine synthesis pw error
  • No hyperammonemia (vs. OTC def.)
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21
Q

What is Diamond-Blackfan anemia?

A

Megaloblastic anemia

  • Defect in erythroid progenitors (onset w/in 1st year)
  • Increased Hb F
  • Short stature, craniofacial abnormalities, UE abnormalities
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22
Q

What does hepcidin do?

A

Inhibits ferroportin

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23
Q

What is Fanconi anemia?

A

DNA repair defect causing bone marrow failure

- Short stature, increased incidence tumors/leukemias, cafe-au-lait spots, thumb/radial defects

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24
Q

What unique labs would be abnormal in PNH?

A 
CD55 neg (DAF)
CD59 neg (MAC inh protein)

Tx is eculizumab (terminal complement inhibitor)

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25
Q

What is the triad a/w PNH?

A

PVC

  • Pancytopenia
  • Venous thrombosis
  • Coombs-neg hemolytic anemia
26
Q

What are warm agglutinins a/w? (IgG)

A
  • SLE
  • CLL
  • Certain drugs (eg alpha-methyldopa)
27
Q

What are cold agglutinins a/w? (IgM)

A
  • Mycoplasma pneumonia

- Mononucleosis

28
Q

How are the transferrin (TIBC) levels altered in anemia of chronic disease?

Pregnancy/OCP use?

A

Decreased (even though serum Fe is low, sequestered to ferritin)

Increased (trying to get more)

29
Q

What enzyme is inhibited in acute intermittent porphyria?

What builds up?

What are the sx? (5 P’s)

A

Porphobilinogen deaminase

  • Porphobilinogen, ALA, coporphobilinogen (in urine)
Painful abd
Port wine-colored urine
Polyneuropathy
Psychological disturbances
Precipitated by drugs, alcohol, starvation
30
Q

What is the most common porphyria?
Enzyme? Buildup?
Major sx?

A

Porphyria cutanea tarda

  • Uroporphyrinogen decarboxylase; uroporphyrin
  • Tea-colored urine; blistering cutaneous photosensitivity
31
Q

Sx of Pb poisoning in kids?

Adults?

A
  • Kids: mental deterioration

- Adults: HA, memory loss, demyelination

32
Q

What disease is defective GP1b?

A

Bernard-Soulier

  • Large platelets (“big suckers”)
33
Q

What disease is defective GP2b3a?

A

Glanzmann thrombasthenia

34
Q

What are the AB’s against in immune thrombocytopenic purpura (ITP)?

A

GP2b3a

  • Splenic macrophage consumption of platelet-AB complex
  • Commonly due to viral illness
  • Increased megakaryocytes on bx
35
Q

How is ADAMSTS 13 related to TTP?

A

ADAMTS 13 is a vWF metalloprotease

- When it’s inhibited or def, decreased breakdown of vWF

36
Q

How would you test for vW dz? (most common inherited bleeding d/o)?
Inheritance pattern?

A
  • Ristocetin (no aggregation)
  • AD

(Increased PTT because vWF carries factor VIII; give desmopressin like w/hemophilia A)

37
Q

List the causes of DIC (there is a mnemonic)

A

STOP Making New Thrombi

Sepsis (gram neg)
Trauma
Obstetric complication
Pancreatitis (acute)
Malignancy
Nephrotic syndrome
Transfusion
38
Q

What is factor V Leiden?

A

Arg506Gln near cleavage site -> mutant factor V can’t be cleaved by protein C -> hypercoagulability (caucasians)

39
Q

In terms of location/spread, what’s the difference b/w Hodgkin and non-Hodgkin lymphoma?

A

HL: Localized, single group of nodes w/contiguous spread

  • Bimodal: young adulthood + > 55 years
  • B sx

NHL: Multiple LN’s involved, extra-nodal involvement common, NONcontiguous spread
- Children or adults

40
Q

List the B cell NHL’s.

List the T cell NHL’s.

A

B cell NHL’s

  • Burkitt
  • Diffuse large B cell
  • Follicular
  • Mantle cell
  • Primary CNS (r/o toxo in AIDS)

T cell NHL’s

  • Adult T cell (HTLV; lytic bone lesions, r/o MM)
  • Mycosis fungoides -> Sezary syndrome
41
Q

In which disease do you see rouleaux formation?

A

Multiple myeloma

42
Q

Sx of MM? (there is a mnemonic)

A 
CRAB
Calcium ^
Renal involvement (recall amyloid)
Anemia (occurs in bones)
Bone lytic lesions; Back pain
  • M spike (usually IgA, 2nd is IgA)
43
Q

What Ig is the M spike in Waldenstrom’s macroglobulinemia?

A

IgM

  • Hyperviscosity syndrome, but no “CRAB” findings
44
Q

What can myelodysplastic syndrome transform to?

A

AML

45
Q

What blood d/o’s are a/w Down syndrome?

A

Acute megakaryoblastic leukemia (AML) before the age of 5, and ALL after the age of 5.

46
Q

What markers are positive in ALL?

A

TdT (marker of pre-T and pre-B cells)
CD10 (marker of pre-B cells)

[t(12:21) has better prognosis]

47
Q

What cells do you see w/CLL/SLL?

A

Chronic lymphocytic leukemia / Small lymphocytic lymphoma

- Smudge cells (Crushed Little Lymphocytes)

48
Q

What is Richter transformation?

A

SLL/CLL transforms (most commonly to DLBCL)

49
Q

What markers are positive in CLL/SLL?

A

CD20

CD5 (normally T cell marker)

50
Q

What is TRAP a/w?

A

Hairy cell leukemia

  • Trap the cells in the spleen (splenomegaly)
  • Can’t be in the marrow (dry tap)
  • Stains TRAP (tartrate-resistant acid phosphatase +)
51
Q

What is pathognomonic for AML on cytology?

A

Auer rods

  • MPO+, but this is mostly for PML
52
Q

*What is a blast crisis a/w?

A

CML: transformation to AML or ALL (anything w/blasts; same dz’s that are in Down syndrome)

53
Q

What test distinguishes CML from a leukemoid rxn?

A

LAP (nl in CML, increased in leukemoid)

54
Q

Where can the Ph c’some be seen besides CML? (rarely)

A

ALL

55
Q

What 3 heme onc diseases can show lytic bone lesions?

A

Multiple myeloma
HTLV -> adult T cell lymphoma
Langerhans cell histiocytosis

56
Q

What are the key points of Langerhans cell histiocytosis? (read)

A
  • DC’s immature and therefore don’t activate T cells
  • Lytic bone lesions, skin rash, recurrent otitis media w/mastoid bone mass
  • S-100+, CD1a+
  • *Birbeck granules (racquetball racket shape)
57
Q

R/f’s for AML?

A

Akylating chemo
Radiation
Myeloproliferative d/o’s
Down syndrome (before age 5; ALL after)

58
Q

List the 4 myeloproliferative d/o’s.

A

Polycythemia vera
Essential thrombocytopenia
Myelofibrosis
(CML)

59
Q

What mutation are myeloproliferative d/o’s a/w?

A

JAK2 mutation (V617F)

60
Q

What does polycythemia vera classically p/w? (1) Rare(1)?

A
  • Intense itching s/p hot shower

- Rare, but classic: severe burning pain and red/blue coloration (erythromelalgia)

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