(released by platelets) - Decreases blood flow - Increases platelet aggregation

Heme onc Flashcards by Dave Ed

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

CD14

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What toxins to eosinophils have that fight helminths?

Major basic protein

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What is mnemonic for fetal erythropoiesis?

Young liver synthesizes blood

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Review fetal erythropoiesis.

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

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

Bradykinin formed from HMWK via kallikrein

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What can activate the intrinsic pw?

“Contact activation”

Collagen, BM, activated platelets, HMWK

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What can activate the extrinsic pw?

Tissue factor

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Function of TXA2?

(released by platelets)

Decreases blood flow
Increases platelet aggregation

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What are acanthocytes and when do you seen them?

RBCs w/spinous-like protrusion

Liver disease
Abetalipoproteinemias

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When do you see basophilic stippling?

Lead poisoning
Sideroblastic anemias
Myelodysplastic syndromes

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When do you seen teardrop cells (dacrocytes)?

Bone marrow infiltration (getting squeezed out)

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When do you see bite cells?

G6PD def.

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What are echinocytes and when do you see them?

“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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When can sickling of RBCs occur in SCD?

Low O2
Low H2O
Acidosis

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When can you see target cells?

“HALT,” said the hunter to his target

Hb C disease
Asplenia
Liver disease
Thalassemia

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

Hb Barts (only gamma)

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

Hb B (some Beta tetramers)

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S/s of Pb poisoning?

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

In X-linked sideroblastic anemia?

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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What type of anemia does orotic aciduria cause?

Megaloblastic

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

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What is Diamond-Blackfan anemia?

Megaloblastic anemia

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

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What does hepcidin do?

Inhibits ferroportin

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What is Fanconi anemia?

DNA repair defect causing bone marrow failure

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

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What unique labs would be abnormal in PNH?

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

Tx is eculizumab (terminal complement inhibitor)

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What is the triad a/w PNH?

PVC - Pancytopenia - Venous thrombosis - Coombs-neg hemolytic anemia

What are warm agglutinins a/w? (IgG)

- SLE - CLL - Certain drugs (eg alpha-methyldopa)

What are cold agglutinins a/w? (IgM)

- Mycoplasma pneumonia | - Mononucleosis

How are the transferrin (TIBC) levels altered in anemia of chronic disease? Pregnancy/OCP use?

Decreased (even though serum Fe is low, sequestered to ferritin) Increased (trying to get more)

What enzyme is inhibited in acute intermittent porphyria? What builds up? What are the sx? (5 P's)

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

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

Porphyria cutanea tarda - Uroporphyrinogen decarboxylase; uroporphyrin - Tea-colored urine; blistering cutaneous photosensitivity

Sx of Pb poisoning in kids? | Adults?

- Kids: mental deterioration | - Adults: HA, memory loss, demyelination

What disease is defective GP1b?

Bernard-Soulier - Large platelets ("big suckers")

What disease is defective GP2b3a?

Glanzmann thrombasthenia

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

GP2b3a - Splenic macrophage consumption of platelet-AB complex - Commonly due to viral illness - Increased megakaryocytes on bx

How is ADAMSTS 13 related to TTP?

ADAMTS 13 is a vWF metalloprotease | - When it's inhibited or def, decreased breakdown of vWF

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

- Ristocetin (no aggregation) - AD (Increased PTT because vWF carries factor VIII; give desmopressin like w/hemophilia A)

List the causes of DIC (there is a mnemonic)

STOP Making New Thrombi ``` Sepsis (gram neg) Trauma Obstetric complication Pancreatitis (acute) Malignancy Nephrotic syndrome Transfusion ```

What is factor V Leiden?

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

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

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

List the B cell NHL's. List the T cell NHL's.

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

In which disease do you see rouleaux formation?

Multiple myeloma

Sx of MM? (there is a mnemonic)

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

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

IgM - Hyperviscosity syndrome, but no "CRAB" findings

What can myelodysplastic syndrome transform to?

AML

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

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

What markers are positive in ALL?

TdT (marker of pre-T and pre-B cells) CD10 (marker of pre-B cells) [t(12:21) has better prognosis]

What cells do you see w/CLL/SLL?

Chronic lymphocytic leukemia / Small lymphocytic lymphoma | - Smudge cells (Crushed Little Lymphocytes)

What is Richter transformation?

SLL/CLL transforms (most commonly to DLBCL)

What markers are positive in CLL/SLL?

CD20 | CD5 (normally T cell marker)

What is TRAP a/w?

Hairy cell leukemia - Trap the cells in the spleen (splenomegaly) - Can't be in the marrow (dry tap) - Stains TRAP (tartrate-resistant acid phosphatase +)

What is pathognomonic for AML on cytology?

Auer rods - MPO+, but this is mostly for PML

*What is a blast crisis a/w?

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

What test distinguishes CML from a leukemoid rxn?

LAP (nl in CML, increased in leukemoid)

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

ALL

What 3 heme onc diseases can show lytic bone lesions?

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

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

- 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)

R/f's for AML?

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

List the 4 myeloproliferative d/o's.

Polycythemia vera Essential thrombocytopenia Myelofibrosis (CML)

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

JAK2 mutation (V617F)

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

- Intense itching s/p hot shower | - Rare, but classic: severe burning pain and red/blue coloration (erythromelalgia)