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Heme Flashcards

1
Q

Autoimmune hemolytic anemia

  • Warm

Cold?

Tx?

A

Warm - IgG - COOMBS + - SLE, CLL, DRugs (methyldopa)

Cold- IgM - mycoplasma, mononucleosis, CLL .

TX BOTH WITH HIGH DOSE STEROIDS

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2
Q

ITP - Cause? wokrup?

Tx algorithm in children vs adults?

A

Anti-G2b3a ab - Post viral. TEST HEP C HIV

Children - Tx only if bleeding (even if plt are 10k. Bruising is fine ) -> steroids IVIG

Adults - Tx if bleeding or if plt less than 30k

TX FOR BOTH - STEROIDS IVIG. NEVER give plt transfusion

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3
Q

TTP - cause tx?

A

Adamts13 vwf multimers

STEROIDS PLASMAPHARESIS.

  • Aka different than ITP, which gives steroids and IVIG
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4
Q

Who to suspect pernicious anemia in?

A

Northern european.

Those w/ other autoimmune conditions - vitiligo - thyroid dysfunction

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5
Q

Tx for b thalassemia?

A

No tx in minor.

Fe is not the problem.

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6
Q

Iron findings in chronic diasese?

A

DEC Iron, INC ferritin, DEC TIBC.

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7
Q

2 porphyria rpesentations

A

Acute intermittent - abd pain, wine urine, AMS - Tx glucose and heme.

Porphyria Cutanea tarda - most common porphyra - Dark urine, photosensitivity rash.

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8
Q

AD blood disorders?x 5

A

H spherocytosis

F5liden

VWD

AT3def

Protein C and S def

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9
Q

H spherocytosis - Inheritance Ways to tests for it? CBC diff findings?

Tx -

A

Test:
Eos5M - flow cytometry
Acid gylcerol lysis
Osmotic fragmetnation

Coombs - . MCHC > 36% - INC RCDW.

Tx splenectomy
- vaccinate + PO penicillin 3-5y. in kids or until adult

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10
Q

vWD - inheritance - Coag findings

ways to tests for it? Tx

A

AD

Normal PT PTT. INC BT.

Ristocetin agglutination Tx

Tx - DDAVP

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11
Q

How can AT3 def be discovered? Inheritance?

A

AD- Normal Pt/PTT time,

Normal Hep dose does not INC PT.

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12
Q

Name AR heme disorderesx 2

Describe

A

Pyruvate KinaseD - DEC ATp = hemolytic in newborn

CBS deficiency - INC Homocysteine, Tall, MR, eye.

Homo - Down
Marfan Up

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13
Q

XR heme disorders?x 2

Describe?

A

XR heme

G6PD - DEC glutathionine , INC risk of oxidative stress. Heinz + ZBite. Screening tests during acute episode is not accurate

Inherited sideroblastic - can be caused by B6 def or ETOH, INH.

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14
Q

Acquired Heme condiitnios x 2

tx for one?

A

PNH - Hemolytic with negative Coombs - CD55. Tx - Eculizamab

Prothrombin def - Acquired hypercoag - INC gene formation

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15
Q

Lab findings/ Tx for antiphospholipid syndrome?

A

Dx - False INC in PTP w/ thrombophilia.

Tx - Aspirin + :LMWH

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16
Q

HIT differentiation?

Dx?
Tx?

A

HIT1 - 2 days - nonimmune - continue hep

HIT 2 - 5-10 days - Dx - Serotonin release assay. D/c Hep and LMWH
Tx- Direct Thrombin or F10 blockeres. Then warfarin

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17
Q

EPO findings in polycythemia?

A

EPO independent - so EPO is suppressed.

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18
Q

What to do with hard solitary nodule in neck of smokier?

A

Biopsy - consider Squamous CCA

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19
Q

Iron def in male, postmenopausal?

lab findings ?

A

Occult blood tests first -

INC RDW >20%. Thalassemia usually does not have this great of an INc in RDW

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20
Q

Types of Hodgkin lymphoma?

Tx complication?: \

A

Nodular sclerosis - female, fibrosis +_ nodules

L! Rich
- best prognosis

Mixed Cellularity - Eois IL5,

L! deplete poor

L! predominant.

ChemoRTX - HIGH RISK OF 2ND MALIGNANCY DUE TO RTX - 30% BY 30 YEAR.

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21
Q

Nonhodkin lymphoma types and mutations?

A

Folliclualr :1418 =- BCL2 - > DLBC. Wax wane

mantle (11:14) - Cyclin D , CD5

Marginal - Chronic Inflam - Hashi, Sjogran,MALTOMA

Burkitts - 8:14 - c-myc - Starry sky - EVC - jaw abd,

DLBC - 14:18 - BCL2 - may be from folliculalr

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22
Q

AML , ALL

CML, CLL

A

M MPO+

L tdt+

ALL - 12:21 - B (Cd10, 20); T - CD1-7, thymic mass

AML - 15:17 - ATRA

CML - Basophilia, often anemic, with HIGh white and plt
Phil 9:22 - AML
BCR-Abl (Tyrosine Kinase) - Tx Imatinib (blcok TK)

CLL -

B cell - cD5 -> DLBCL
smudge cell

Hairy = TRAP, dry tap, fiobrosis
Cladribine 2-CDA

T - ATLL - rash + lytic (HTLV1)
Mycosis fungoides - ctaneousl . Pautrier microabscess. Sezary in blood - cerebroform lesions.

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23
Q

MGUS -

Waldenstrom macroglobulinemia

MM - dx

A

MGUS - Asx M spike, less than 10% plasma cells.

Walden- M spike IgM, Hyperviscosity. NO LYTIC LESIOSN

M spike, Fried Egg - IgG55% IgA 25% - bence jones paraprotein, IL6!!
Dx - serum immunoelectropheresis before you marrow biopsy

24
Q

Langerhan - presentation - Lab findings, microscopy

A

langerhans - Lytic bone + rash (aslto T cCLL) - recurrent otitis media.
S100+, CD19+, Birbeck granules

25
Q

Antimetabolites

MTX

5FU

Cytarabine

Azathiprine/6mp

Cladribine (2CDA)

Hydroxyurea

A

MTX - Folate analog - Leucovorin - Tox myelosuppression, STOMATITIS , macrovesciular hepatotox

5FU - pyrimidine analgo - Uridine Rescue

Cytarabie - panCYTOPENIA

6mp - Prine - Tox w/ Alllopurinal (metabolized by XO)

Cladribine - hairy ccell DOC

Hydroxyurea - Block ribonucleotide reductase - Bone marrow tox - GI upset

26
Q

Antibiotic heme drugs

A

Actinomycin - Intercalate - kids ACT OUT (Wilms, Ewings, Rhabdomyosarcoma

Bleomycin - INC free radical - Tx - testicular CA, hodgkins
Tox - pulm fibrosis , skin changes

Doxorubicin - Dexrazoxone to prevent cardiotox. ALOPEICA .

27
Q

Alkylating agents

Busulfan

Cis/Carbo PLATIN

Cyclophosphamide

nitrosurea (STINES)

A

Alkylating agents

Busulfan - Pulm fibrosis, HYPERPIGGEMTNATIOn, Severe myelosuppression

Cis/Carboplatin - AMIFOSTINE + IV hydration NEPHROTOX, acoustic neuromas.

CyclophosphaMESNA - Tox hemorrhagic cystitis, CA, SIADH.

Nitrosurea stines) - CROSS BBB. GGM Tx. CNS tox

28
Q

Topoisomerases -

A

Irinotecan/topotecan - Topo 1 - single strand

EToposide, Teniposide - Topo 2

29
Q

M phase blocker

A

Taxols - Hyperstabilize - Tx ovarian Breast

Vincristine/bastine - Blocks tubule formatin - NEUROTOX (paralytic ileus);

30
Q

F10 inhibitors can be used for?

A

Apixaban,
Rivaroxaban,
Fondiparanox

F10 blockers

PO, no monitoring, no reversing

Can be used for acute DVT and PE tx! As well as maintenance

31
Q

F2 can be used in?

A

Pt w/ T2 HIT

Argatroban, Bival.

32
Q

B-CLL tx?

A

Rituximab - Anti CD 20

OR

CYCLOPHOSHAMIDE!

or

Fludarabine

33
Q

Myelodysplastic syndrome - presentation and histology findings

A

Pancytopenias and variations.

Histo: Ovalo Macroytes and HYPOSEGMENTED N!

34
Q

Pernicious anemia - what to dx?

A

Anti IF and Anti Parietal cells -

SCHILLING TEST IS NEVER THE RIGHT ANSWER.

35
Q

PNH - most likely cause of death?

Best initial tx?
Other tx?
Cure?

A

Thrombosis (Large vessel and mesenteric/hepatic vein)

Initial - steroids. Next Eculizumab

Cure - Transplant bone marrow.

36
Q

ALL tx -

A

Intrathecal methotrexate!!!?

37
Q

Acute Leukostasis Reaction

someone come sin with WBC of 200K etc

What to do?

A

Leukapheresis - need to DEC the WBC stat

Can find out the dx later.

38
Q

Tx of Myelodysplastic Syndrome?

A

Azacitidine, Lenalidomide

39
Q

What ppx is indicated in CLL?

A

PCP

40
Q

Nonhodgkins diagnosis?

A

Excision biopsy. NOT NEEDLE ASPIRATION, because the individual cytes are fine, so you need to see architecture.

41
Q

Ways to scan for cardiotox in doxorubicin

A

Sure ECHo is fine, but MUGA/NUCLEAR VENTRICULOGRAM is best. Cannot use adriamycin if EF is less than 50%

42
Q

Tx for Waldenstroms Macroglobulinemia?

A

Phlasmapheresis is best initial tx.

43
Q

Best INITIAL tx for ITP?

A

Steroids

IVIG after

44
Q

MGUS dx? What workup must be done?

A

dx involves rule out of MM – Skeletal bone scan, with possible bone marrow biopsy

45
Q

Fanconia anemia

A

– inherited – Chromosomlal breaks – Pancytopenia, short stature, abnormal thumbs, café au lai/hypo/hyperpigmentation. Tx stem cell tx.

46
Q

PNH associations? Concerns?

A

associated with hypercoag state (VENOUS THROMBOSIS) + cytopenias.

47
Q

Anticoagulation in pt w/ renal insufficiency (GFR less than 30

A

MUST GIVE UNFRACTIONATED HEPARIN – Cannot give LMWH, fondaparainux, and rivaroxaban to pt w/ renal problems. Bridge to Warfarin.

48
Q

Blood transufion reactions –

Immedaite
1 hr
1-6 hr
2-3 hr 
6h 
2-10 d
A

Anaphylactic – anti IgA

Acute hemolytic within 1 hr

Urticarial 2-3hr urticarial, flushing angioedema IgE ab and mast cell activation.

Febrile nohemolytic 1-6 hr fevers chiklls: cytokines ; , hemoglobinurkia, RF, DIC, ABO incompatability.

Transfusion related acute lung injury 6hr, donor anti leukocyte ab with respiratory distress.

Delated hemolytic 2-10 days after anamnestic ab response.

49
Q

Schistocyties in scleroderma pt

A

Scleroderma renal crisis – sudden onset renal failure w/ microangiopathic hemolytic anemia

50
Q

Arterial clot algorithm

A

IV hep, then IR intraARTERIAL fibfrinolysis or embolectomy

51
Q

Sickle cell in kids, what ppx

A

Give 3 vacciens, penicillin ppx until 5.yo. (similar to splenectomy and rheumatic)

52
Q

Neutropenia/neutropenic fever thresholds/critera

A

ANC less than 1500; severe is ANC less than 500. Tx with antipseudomonal if febrile.

53
Q

How to confirm ALL

A

must bone marrow biopsy to show >20/25% lymphoblasts in amrrow. Blood smear is not a confirmation.

54
Q

SS which bug do you see in osteo

A

– Staph> Salm

55
Q

Febrile nonhemolytic reaction, what to do if you ened to give blood and in future cases. Which pt do you do this with ppx?

A

Leukoreduction – get rid of the cytokines and remnant white blood cells that cause the febrile rnonhemolytic rx.

Do this ppx in chronically transfused and CMV seronegative pt at risk (aids tx).

56
Q

When do yu wash RBC?

A

– IgA def, complement hemolytic anemia.

57
Q

When do you irradiate RBC

A

Bone marrow tx, immunodeficiency, blood from 1st or 2nd degree rlatives.

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