Heme Flashcards
Autoimmune hemolytic anemia
- Warm
Cold?
Tx?
Warm - IgG - COOMBS + - SLE, CLL, DRugs (methyldopa)
Cold- IgM - mycoplasma, mononucleosis, CLL .
TX BOTH WITH HIGH DOSE STEROIDS
ITP - Cause? wokrup?
Tx algorithm in children vs adults?
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
TTP - cause tx?
Adamts13 vwf multimers
STEROIDS PLASMAPHARESIS.
- Aka different than ITP, which gives steroids and IVIG
Who to suspect pernicious anemia in?
Northern european.
Those w/ other autoimmune conditions - vitiligo - thyroid dysfunction
Tx for b thalassemia?
No tx in minor.
Fe is not the problem.
Iron findings in chronic diasese?
DEC Iron, INC ferritin, DEC TIBC.
2 porphyria rpesentations
Acute intermittent - abd pain, wine urine, AMS - Tx glucose and heme.
Porphyria Cutanea tarda - most common porphyra - Dark urine, photosensitivity rash.
AD blood disorders?x 5
H spherocytosis
F5liden
VWD
AT3def
Protein C and S def
H spherocytosis - Inheritance Ways to tests for it? CBC diff findings?
Tx -
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
vWD - inheritance - Coag findings
ways to tests for it? Tx
AD
Normal PT PTT. INC BT.
Ristocetin agglutination Tx
Tx - DDAVP
How can AT3 def be discovered? Inheritance?
AD- Normal Pt/PTT time,
Normal Hep dose does not INC PT.
Name AR heme disorderesx 2
Describe
Pyruvate KinaseD - DEC ATp = hemolytic in newborn
CBS deficiency - INC Homocysteine, Tall, MR, eye.
Homo - Down
Marfan Up
XR heme disorders?x 2
Describe?
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.
Acquired Heme condiitnios x 2
tx for one?
PNH - Hemolytic with negative Coombs - CD55. Tx - Eculizamab
Prothrombin def - Acquired hypercoag - INC gene formation
Lab findings/ Tx for antiphospholipid syndrome?
Dx - False INC in PTP w/ thrombophilia.
Tx - Aspirin + :LMWH
HIT differentiation?
Dx?
Tx?
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
EPO findings in polycythemia?
EPO independent - so EPO is suppressed.
What to do with hard solitary nodule in neck of smokier?
Biopsy - consider Squamous CCA
Iron def in male, postmenopausal?
lab findings ?
Occult blood tests first -
INC RDW >20%. Thalassemia usually does not have this great of an INc in RDW
Types of Hodgkin lymphoma?
Tx complication?: \
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.
Nonhodkin lymphoma types and mutations?
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
AML , ALL
CML, CLL
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.
MGUS -
Waldenstrom macroglobulinemia
MM - dx
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
Langerhan - presentation - Lab findings, microscopy
langerhans - Lytic bone + rash (aslto T cCLL) - recurrent otitis media.
S100+, CD19+, Birbeck granules
Antimetabolites
MTX
5FU
Cytarabine
Azathiprine/6mp
Cladribine (2CDA)
Hydroxyurea
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
Antibiotic heme drugs
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 .
Alkylating agents
Busulfan
Cis/Carbo PLATIN
Cyclophosphamide
nitrosurea (STINES)
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
Topoisomerases -
Irinotecan/topotecan - Topo 1 - single strand
EToposide, Teniposide - Topo 2
M phase blocker
Taxols - Hyperstabilize - Tx ovarian Breast
Vincristine/bastine - Blocks tubule formatin - NEUROTOX (paralytic ileus);
F10 inhibitors can be used for?
Apixaban,
Rivaroxaban,
Fondiparanox
F10 blockers
PO, no monitoring, no reversing
Can be used for acute DVT and PE tx! As well as maintenance
F2 can be used in?
Pt w/ T2 HIT
Argatroban, Bival.
B-CLL tx?
Rituximab - Anti CD 20
OR
CYCLOPHOSHAMIDE!
or
Fludarabine
Myelodysplastic syndrome - presentation and histology findings
Pancytopenias and variations.
Histo: Ovalo Macroytes and HYPOSEGMENTED N!
Pernicious anemia - what to dx?
Anti IF and Anti Parietal cells -
SCHILLING TEST IS NEVER THE RIGHT ANSWER.
PNH - most likely cause of death?
Best initial tx?
Other tx?
Cure?
Thrombosis (Large vessel and mesenteric/hepatic vein)
Initial - steroids. Next Eculizumab
Cure - Transplant bone marrow.
ALL tx -
Intrathecal methotrexate!!!?
Acute Leukostasis Reaction
someone come sin with WBC of 200K etc
What to do?
Leukapheresis - need to DEC the WBC stat
Can find out the dx later.
Tx of Myelodysplastic Syndrome?
Azacitidine, Lenalidomide
What ppx is indicated in CLL?
PCP
Nonhodgkins diagnosis?
Excision biopsy. NOT NEEDLE ASPIRATION, because the individual cytes are fine, so you need to see architecture.
Ways to scan for cardiotox in doxorubicin
Sure ECHo is fine, but MUGA/NUCLEAR VENTRICULOGRAM is best. Cannot use adriamycin if EF is less than 50%
Tx for Waldenstroms Macroglobulinemia?
Phlasmapheresis is best initial tx.
Best INITIAL tx for ITP?
Steroids
IVIG after
MGUS dx? What workup must be done?
dx involves rule out of MM – Skeletal bone scan, with possible bone marrow biopsy
Fanconia anemia
– inherited – Chromosomlal breaks – Pancytopenia, short stature, abnormal thumbs, café au lai/hypo/hyperpigmentation. Tx stem cell tx.
PNH associations? Concerns?
associated with hypercoag state (VENOUS THROMBOSIS) + cytopenias.
Anticoagulation in pt w/ renal insufficiency (GFR less than 30
MUST GIVE UNFRACTIONATED HEPARIN – Cannot give LMWH, fondaparainux, and rivaroxaban to pt w/ renal problems. Bridge to Warfarin.
Blood transufion reactions –
Immedaite 1 hr 1-6 hr 2-3 hr 6h 2-10 d
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.
Schistocyties in scleroderma pt
Scleroderma renal crisis – sudden onset renal failure w/ microangiopathic hemolytic anemia
Arterial clot algorithm
IV hep, then IR intraARTERIAL fibfrinolysis or embolectomy
Sickle cell in kids, what ppx
Give 3 vacciens, penicillin ppx until 5.yo. (similar to splenectomy and rheumatic)
Neutropenia/neutropenic fever thresholds/critera
ANC less than 1500; severe is ANC less than 500. Tx with antipseudomonal if febrile.
How to confirm ALL
must bone marrow biopsy to show >20/25% lymphoblasts in amrrow. Blood smear is not a confirmation.
SS which bug do you see in osteo
– Staph> Salm
Febrile nonhemolytic reaction, what to do if you ened to give blood and in future cases. Which pt do you do this with ppx?
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).
When do yu wash RBC?
– IgA def, complement hemolytic anemia.
When do you irradiate RBC
Bone marrow tx, immunodeficiency, blood from 1st or 2nd degree rlatives.
