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Mksap > Heme/onc > Flashcards

Heme/onc Flashcards

1
Q

PRCA

A
  • Pure red cell aplasia
  • Autoimmunity or viral->T-cell autoimmunity (pregnancy, thymoma, malignancy), parvoB19
  • In contrast to pancytopenia=bone marrow failure, cirrhosis, hypersplenism, aplastic anemia, MDS
  • inadequate reticulocyte response
  • bone marrow for dx and to rule out lymphoproliferative (CLL, non-hodgkin lymphoma)
  • Large granular lymphocytosis->associated w/ PRCA and RA->lymphocyte CD 57+ ->treat w/ methotrexate->overlap w/ Felty syndrome (RA, splenomegaly, neutropenia)
  • Treatment: prednisone, cyclosporine, cyclophosphamide.
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2
Q

Aplastic anemia

A
  • Pancytopenia
  • Hypocellular bone marrow (toxic, viral, AUTOIMMUNE
  • Transient->NSAIDs, beta lactams, antiepileptics, psychotropics.
  • Treatment->cyclosporine; antithymocyte globulin; allogeneic HSCT (erythrocytes lack membrane proteins CD55/59 which is needed to stabilize complement->results in episodic hemolysis.
  • 10% develop MDS or AML w/in 10 years.
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3
Q

Neutropenia

A
  • African/Middle Eastern often have w/o significance
  • Acute HIV, CMV, EBV, Rickettsial
  • NSAIDs, carbamazepine, phenytoin, PTU, cephalosporins, TMP-SMX, psychotropics
  • SLE, RA
  • Vitamin B/folate deficiency
  • treatment: underlying cause, granulocyte CSF
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4
Q

MDS

A
  • Hypercellular bone marrow
  • macrocytic anemia/pancytopenia w/ b12/folate deficiency ruled out.
  • concern for transformation to AML
  • Treatment: sometimes erythropoiesis stimulating agents, HSCT, hypomethylating chemo (azacytidine, decitabine); in mild disease->no treatment
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5
Q

Myeloproliferative neoplasms/disease (MPN/MPD)

A 
CML (Philadelphia chrom +)
Polycythemia Vera
Essential thrombocythemia
Primary myelofibrosis
Hypereosinophilic syndromes (HESs)

S/S: thrombosis, massive splenomegaly, constitutional (fevers/chills/night sweats, wt loss)

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6
Q

Chronic Myeloid Leukemia (CML)

A

Granulocytic leukocytosis
Sometimes confused w/ leukemoid reaction (rarely >50k; blasts/basophils less likely)
Philadelphia chromosome [t(9;22)]->BCR-ABL->encodes tyrosine kinase
CML–>AML (80%); ALL (20%)

Treatment: TKIs->Imatinib (1st gen); nilotinib/disatinib (2nd gen)
SE: prolong QT; disatinib->pericardial/pleural effusions and pulm HTN; not safe in
Pregnancy

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7
Q

Polycythemia Vera

A

Myeloid/erythroid disorder
JAK2 mutation
Hgb > 18.5 (men) or 16.5 (women)
Pruritis after shower; TIA; DVT/PE; splenomegaly; plethora; “erythromelalgia”; low epo; basophilia; leukocytosis
AML (10%)
Treatment: low-dose aspirin; phlebotomy (goal hct

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8
Q

Essential Thrombocythemia

A

Plt >600k
JAK2 (50%)
Low-risk: aspirin or observation
High-risk: hydroxyurea; plateletpheresis in emergent cases

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9
Q

Primary Myelofibrosis

A

Etiology: clonal myeloid disorder w/ abnormal, proliferating megakaryocytes producing excess fibroblast growth factor->marrow fibrosis w/ extramedullary hematopoiesis.
Often “dry tap”
Elevated LDH and Uric acid
JAK2 (50%)
HUGE spleen
Treatment: Allogeneic HSCT for younger pt’s; o/w palliative

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10
Q

Eosinophilia and HES

A

“CHINA”: collagen (eosinophilic granulomatosis “Churg-Strauss”), Helminth, Idiopathic, Neoplasia (lymphomas), allergy/atopy/asthma (drugs->carbamazepine, sufonamides)
HES: eosinophilia > 1500 w/ tissue infiltration; primary vs reactive; primary->PDGFR alpha or beta activation
Treatment: primary=glucocorticoids (lyse eosinophils) vs imatinib

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11
Q

Acute Myeloid Leukemia

A

MDS vs MPN vs de novo
Mean age 67
>20% myeloblasts blood or bone; Ill in days/weeks
Pancytopenia
Adults 90% AML vs 10% ALL->reverse for children/adolescents
Auer rods
APL–>DIC, promyelocytes, prominent Auer rods, FISH for t(15;17)->ATRA
Treatment: Induction w/ cytosine arabinoside (cytarabine) and an anthracycline; >50k myeloblasts=hyperleukocytosis syndrome->leukapharesis

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12
Q

Acute Lymphoblastic Leukemia

A

CNS involvement
>25% lymphoblasts->usually deoxynuceotidyl transferase (+) and myeloperoxidase (-)
B-cell, Philadelphia chrom, MLL gene rearrangement, hypodiploidy–>poor prognosis
Treatment: Younger adults=asparaginase vs allo HSCT; if Philadelphia +–>TKI (disatinib)

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13
Q

Hematopoietic Growth Factors

A

G-CSF–>stimulate neutrophils; usually pegylated w/ activity for several weeks
Recombinant erythropoietin: anemia of CKD in dialysis or pre-dialysis if iron/B12/folate replete–>can lead to arterial/venous thrombosis (target hgb ~11)

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14
Q

Hematopoietic Stem Cell Transplantation (HSCT)

A

AA, high-risk MDS, acute leukemias

GVHD–>high-dose glucocorticoids

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15
Q

Plasma Cell Dyscrasias (PCDs)/monoclonal gammopathies

A

Etiology: production of monoclonal antibody (M protein); M protein consists of heavy chain (IgG, IgA, IgD, IgM) complexed w/ kappa or lambda light chain or free light chain; can be plasma cell vs B-cell disorders.
MGUS
MM
Immunoglobulin light-chain (AL) amyloidosis
Waldenstrom Macroglobulinemia
Cryoglobulinemia

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16
Q

Testing for monoclonal gammopathies

A

Lytic bone lesions, Age-inappropriate bone loss, peripheral neuropathy, kidney disease, anemia.
Tests: SPEP/UPEP, serum/urine immunofixation (determine isotype), serum FLC (most sensitive for monoclonal FLC gammopathies)
Interpretation: ratio of kappa/lambda–>increased component (“involved” light chain) indicates PCD

17
Q

Multiple Myeloma

A

Smoldering myeloma= no s/s; usually dx after elevated “total protein” on routine labs; M-protein >3g/dL or clonal plasma cells >10% bone marrow–>usually progresses to MM/AL Amyloidosis in 5-10 years–>monitor q3-6 months (CBC, calcium, Cr, M protein)
IgG or IgA (IgM=WM)
MM: >60% bone marrow, involved/uninvolved ratio >100, calcium >11, >1 lytic bone lesion.
Blood smear=rouleaux formation
Lytic bone lesions, bone pain, hypercalcemia, respiratory infections, kidney dysfunction from cast nephropathy (myeloma kidney)
Treatment: HSCT w/ high dose melphalan; Zoledronic acid/pamidronate q3-4 weeks

18
Q

Immunoglobulin Light-Chain Amyloidosis

A

Extracellular deposition of LMW proteins in beta-pleated sheet configuration
AL, Hereditary, AA, Age-related, Dialysis related
AL=most common; monoclonal free lambda/kappa FLC; PCD related (MGUS/MM); dx abd fat pad aspirate/BM Bx; “apple-green birefringence under polarized light w/ Congo red stain; f/u tests=trop T, N-terminal proBNP, CXR, echo, ECG, LFTs, abd US, PT/PTT (if abnormal, factor X)
AA=Amyloid A protein; RA, IBD, familial Mediterranean fever, chronic infection
Dialysis related=Beta2 microglobulin
Hereditary=mutated transthyretin (TTR)

19
Q

Waldenstrom Macroglobulinemia

A

Indolent B-cell non-Hodgkin lymphoma producing IgM k or lambda M protein.
“B” symptoms (drenching sweats, fever, wt loss), bleeding diathesis, often distal sensorimotor polyneuropathy
Bleeding diathesis=medical emergency and requires immediate plasmapheresis.
Fundoscopy may reveal hyperviscosity related findings–>dilated retinal veins, papilledema, flame hemorrhages.

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