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Study Period Targeted Review > Heme Onc > Flashcards

Heme Onc Flashcards

1
Q

ALL (acute lymphoblastic leukemia)

A

common in kids
associated w/ Down syndrome
TdT+ (marker of pre-T and pre-B cells), CD10+
most resposive to therapy, may spread to CNS and testes
t(12;21) = better prognosis

B-ALL and T-ALL:
T-ALL (15-17%) associated w/ mediastinal mass (presents as SVC-like syndrome; may compress esophagus, trachea –> dysphagia, dyspnea, stridor). B-ALL (70-80%) p/w fever, malaise, bleeding, HSM

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2
Q

Chronic lymphocytic leukemia/small lymphocytic lymphoma

A 
Elderly, most common adult leukemia
CD20/23/5+ B cell neoplasm
Slow progression, often asymp.
Smudge cells ("Crushed Little Lymphocytes")
Richter transformation into DLBCL
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3
Q

Hairy cell leukemia

A

Old men, TRAP+ (“it’s a trap!”)
Fuzzy on LM
Marrow fibrosis (dry tap on aspiration), massive splenomegaly, pancytopenia

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4
Q

AML (acute myelogenous leukemia) and APML (acute promyelocytic leukemia)

A

Older people 65 yo
Auer rods pathognom, (myelo)peroxidase+
Increased myeloblasts on periph smear
Risks: prior alkylating chemo, XRT, myeloproliferative disorders, Down syndrome

APML: t(15;17) forming PML/RARa fusion product that codes for abnormal retinoic acid receptor. Responds to all-trans retinoic acid (vit A), inducing diff’ation of promyelocytes; abundant Auer rods; DIC common

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5
Q

CML

A

Peak 45-85 yo, median 64 yo
Philly chromosome (t(9;22), BCR-ABL)
Dysregulated granulocyte prodxn, splenomegaly
Can transform into AML or ALL (blast crisis)
LAP is low (vs. leukemoid rxn)
Tx w/ tyr kinase inhibitors like imatinib

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6
Q

Factor V Leiden deficiency

A

Inherited mutation to factor V that makes it resistant to degradation by protein C –> most common cause of inherited hypercoagulability in Caucasians. Increased risk of thrombotic events (DVT/PE, cerebral vein thromboses, recurrent pregnancy loss)

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7
Q

Protein C or S deficiency

A

Inability to inactivate factors 5 and 8

Protein C deficiency –> increased risk of warfarin skin necrosis (because warfarin inactivates vit K dependent factors 2, 7, 9, 10, C, S - and C is first to fall with initiation of therapy d/t short half life, causing transient hypercoagulable state –> microthrombi, skin necrosis

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8
Q

Hodgkin lymphoma + subtypes

A 
Localized group of nodes
Contiguous spread
Better prognosis overall than NHL
Reed-Sternberg cells (owl eyes)
Bimodal (young adults and > 55 yrs); more common in men except NS type
EBV-associated

Nodular sclerosing (NS) - most common
Lymphocyte rich - best prognosis
Mixed cellularity - eosinophils, immunocomp
Lymphocyte depleted - immunocomp

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9
Q

Non-Hodgkin lymphoma

A

Multiple LNs involved & extranodal involvement common
Noncontiguous spread
Mostly B cells involved; few T cell lineage
Adults and children affected
may be HIV/autoimmune-associated

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10
Q

Pyruvate kinase deficiency

A

AR disorder; results in decreased ATP –> rigid RBCs –> extravascular hemolysis / hemolytic anemia in a newborn

Increases levels of 2,3 BPG to reduce Hb affinity for O2 (release more O2 to tissues)

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11
Q

Pure red cell aplasia

A

Severe hypoplasia of erythroid marrow elements (causing anemia & low retics), but normal thrombopoiesis & granulopoiesis. Associated w/ thymoma, lymphocytic leukemias, & Parvo B19

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12
Q

Multiple myeloma vs. Waldenstrom macroglobulinemia

A

MM: Plasma cell cancer (fried egg appearance) arising in BM; produces lots of (mostly) IgG and (some) IgA. CRAB: hyperCa, Renal involvement, Anemia, Bone lytic lesions/Back pain.

WM: M spike (IgM) –> hyperviscosity syndrome (blurred vision, Raynaud’s). No CRAB sx.

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13
Q

ALK

A 
Oncogene; receptor tyr kinase
Lung adenocarcinoma ("Adenocarcinoma of the Lung Kinase = ALK")
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14
Q

BCR-ABL

A

Oncogene; tyrosine kinase

CML, ALL

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15
Q

BCL-2

A

Oncogene; antiapoptotic molecule

Follicular and DLBC lymphomas

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16
Q

BRAF

A

Oncogene; serine/threonine kinase (cytoplasmic signal transducer protein)
Melanoma (V600E), NHL, papillary thyroid carcinoma

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17
Q

c-KIT

A

Oncogene; cytokine receptor

GIST

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18
Q

c-MYC

A 
Oncogene; transcription factor
Burkitt lymphoma (t(8;14))
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19
Q

HER2/neu (c-erbB2)

A

Oncogene; receptor tyrosine kinase

Breast and gastric carcinomas

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20
Q

JAK2

A

Oncogene; cytoplasmic (non-receptor) tyrosine kinase

Chronic myeloproliferative disorders e.g. polycythemia vera

21
Q

KRAS

A

Oncogene; GTPase

Colon, lung, pancreatic cancer

22
Q

MYCL1

A

Oncogene; transcription factor

Lung tumor

23
Q

N-myc (MYCN)

A

Oncogene; transcription factor

Neuroblastoma

24
Q

RET

A

Oncogene; receptor tyr kinase

MEN 2A/2B, papillary thyroid carcinoma

25
APC
``` Tumor suppressor; negative regulator of beta-catenin/WNT pathway Colorectal cancer (FAP) ```
26
BRCA1/2
Tumor suppressor; DNA repair protein | BReast, ovarian and pancreatic CAncer (BRCA)
27
MEN1
Tumor suppressor; Menin | Multiple Endocrine Neoplasia 1 (pituitary, pancreas, parathyroid)
28
NF1
Tumor suppressor; neurofibromin (Ras GTPase activating protein) NeuroFibromatosis 1
29
NF2
Tumor suppressor; Merlin (schwannomin) protein | NeuroFibromatosis 2
30
PTEN
Tumor suppressor; negatively regulates P13k/AKT pathway | Breast, prostate, endometrial cancer
31
Rb
Tumor suppressor; inhibits EF2, blocks G1 --> S phase | RetinoBlastoma, osteosarcoma
32
TP53
Tumor suppressor; p53, activates p21, blocks G1 --> S phase | Most human cancers, Li-Fraumeni syndrome (sarcoma, breast, leukemia, adrenal malignancies at early age)
33
TSC1
Tumor suppressor; hamartin protein | Tuberous sclerosis
34
TSC2
Tumor suppressor; tuberin protein | Tuberous sclerosis
35
VHL
Tumor suppressor; inhibits inducible factor 1a | Von Hippel-Lindau disease
36
WT1
``` Tumor suppressor; TF that regulates urogenital development Wilms Tumor (nephroblastoma) ```
37
TTP
ADAMSTS13 inhibition or deficiency --> unable to degrade vWF multimers. Large multimers cause increased platelet adhesion/aggregation/thrombosis. FAT RN (w/ big TT's): Fever, mahA, Thrombocytopenia, Renal failure, Neurologic symptoms Labs: high BT; schistocytes & high LDH (hemolytic anemia). Tx w/ plasmapheresis, steroids Adults: more neuro sx (TTP); kids: more renal sx (HUS) - on a spectrum
38
ITP
Anti-GpIIbIIIa antibodies --> splenic macrophages consume plt-antibody complex. Will have no fever, normal PT and PTT, high BT, high megakaryocytes on BM biopsy. Tx: steroids, IVIG, rituximab or splenectomy if refractory
39
Acute Intermittent Porphyria
Deficient porphobilinogen deaminase --> porphobilinogen and ALA build up. No skin photosensitivity; painful abdomen, port-wine colored urine that darkens when exposed to sunlight, polyneuropathy, psych disturbances. Precipitated by drugs, alcohol, starvation. Tx: hemin and glc to inhibit ALA synthase
40
Porphyria cutanea tarda
Deficient uroporphyrinogen decarboxylase --> build up of uroporphyrin (tea colored urine). Causes blistering cutaneous photosensitivity, hyperpigmentation. Most common, assoc w/ alc consumption and Hep C
41
Paroxysmal nocturnal hemoglobinuria
PIGA gene defect leads to uncontrolled c' mediated hemolysis; lack of CD55/CD59 c' inhibitor proteins = RBC not protected against c', so MAC forms. triad: hemolytic anemia (hemoglobinuria), pancytopenia, thrombosis in atypical sites. Chronic hemolysis can cause iron deposition in kidney (hemosiderosis). Tx: eculizumab (prevents MAC formation)
42
Main defect in: - vWF disease - Bernard-Soulier - Glanzmann thrombasthenia - ITP
- vWF disease: lack of vWF = can't bind vWF to platelet to form plt plug. No ristocetin agglut (ristocetin brings Gp1b and vWF together) - Bernard-Soulier: decreased Gp1b, large plts (Big Suckers); no ristocetin agglut - Glanzmann: Defective GpIIbIIIa, can't form plt plug. Normal risto but abnormal plt to plt clumping - ITP: antibodies against GpIIbIIIa; increased megakaryocytes on BM biopsy
43
General lab findings with intravascular hemolysis
- increase bili, Hb, LDH (all released when RBCs lyse) - increased reticulocyte count - decreased haptoglobin (binds free Hb that spills out of lysed RBCs to promote uptake by reticulo-endothelial system)
44
Langerhans cell histiocytosis
Kids w/ lytic bone lesions, rash or recurrent otitis media w/ mastoid mass. Langerhans cells proliferate but are immature and can't effectively stimulate T cells via Ag presentation. Cells are S100+, CD1a+, Birbeck (tennis racket/rod shaped) granules on EM
45
Sickle cell trait characteristics
Usually asymptomatic but may have priapism, increased UTI risk, hematuria. Relative protection from P. falciparum malaria
46
Hereditary spherocytosis + Tx
Extravascular hemolysis d/t defective RBC membrane proteins (ankyrin, spectrin). AD (structural defect). Small, round RBCs w/ less SA and no central pallor (high MCHC and RDW). Hemolysis/premature removal by spleen is what causes sx (splenomegaly); thus splenectomy is curative (often done w/ cholecystectomy bc assoc w/ cholecystitis, jaundice)
47
Treatment for DVT (prevention/short-term and maintenance), imaging
- Hep or LMWH for prophylaxis and acute management - Oral anticoags (warfarin, rivaroxaban) for long-term maintenance - Imaging: compression US w/ Doppler
48
HIT/treatment for HIT
Heparin induced thrombocytopenia: IgG forms against heparin-bound PF4; these complexes activate plts --> thrombosis and thrombocytopenia. Can use direct IIa inhibitors (Bivalrudin, Argatroban, Dabigatran) - when Hep is BAD for the patient
49
Polycythemia vera
Primary polycythemia: increased RBCs/RBC mass; intense itching after hot shower; erythromelalgia d/t episodic clots in extremity vessels; DECREASED EPO (vs. 2ndary polycythemia). Tx: phlebotomy, hydoxyurea, ruxolitinib (JAK inhibitor)

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