Flashcards in Hematology/Oncology Deck (109):
-Erythrocytosis = polycythemia = increased RBCs
-Anisocytosis = varying sizes
-Poikilocytosis = varying shapes
-Reticulocyte = immature RBC
supplies the biconcavity/flexibility of RBCs
1/3 of platelets are stored in?
Spleen (lifespan = 8-10 days)
Causes of polycythemia/erythrocytosis?
-Any time have increased EPO (hypoxia, renal cell carcinoma, HCC, pheochromocytoma, hemangioblastoma)
-Down's babies at birth
-Polycythemia vera --> have increased RBCs, but normal EPO
=inactivated X in neutrophils
-->seen in all women + KF men
When do you see hypersegmented polymorphic neutrophils?
-Vitamin B12/Folate deficiency
Neutrophil chemotactic factors?
CD14 = cell marker for?
Causes of Eosinophilia?
-Asthma (and Churg-Strauss)
-Collagen Vascular diseases
-Addison's (adrenal insufficiency)
-Acute Interstitial Nephritis
-brain = microglia
-tissue = macrophages
-liver = kupffer cells
-joints = A-cells
Mast cells vs Basophils?
Both are similar, mediate allergic rxn
-Basophils in Blood
-Mast cells in tissue
-mast cells are involved in type 1 hypersensitivity rxns
-dendritic cells on skin and mucous membranes
-act as professional APCs
-possess Birbeck granules = racquet-shaped intracytoplasmic granules
Main inducers of primary antibody response?
Dendritic cells = professional APCs
Plasma cells (B cells differentiate into plasma cells; plasma cells produce lots of antibody specific to a particular antigen)
plasma cell neoplasm?
Multiple Myeloma --> make a whole bunch of one particular type of B cell, all making one type of antibody (so see monoclonal antibody spike)
on ALL T-cells
-->Th also have CD4
-->Cytotoxic also have CD8
anti-AB antibodies (Ig type?)
-IgM --> don't cross placenta
anti-Rh antibodies (Ig type?)
=IgG --> can cross placenta and cause hemolytic disease of newborn (erythroblastosis fetalis) if fetus is Rh+
Blood type A (example)
-have A antigen on RBC surface
-B antibody in plasma
-how to prevent?
-type 2 hypersensitivity rxn
-cause: Rh- mother exposed to Rh+ fetal blood during delivery; makes anti-Rh IgG --> can cross placenta in subsequent pregancies, causing hemolytic disease of newborn in next fetus who is Rh+
-Symptoms in infant: hepatosplenomegaly, severe anemia, jaundice, demise/death
-Treat/Prophylaxis = give mother Rhogam = Rho (D) Ig at first delivery to prevent all future erythroblastosis
Deficiency of Factor VIII?
Deficiency of Factor IX?
Vitamin K deficiency?
decreased synthesis of "diSCo 1972"
-proteins C and S
-Factors X, IX, VII, II (10, 9, 7, 2)
*Vitamin K normally catalyzes carboxylation of glutamic acid residues on proteins involved with blood clotting
Vitamin K antagonist?
-->acts by inhibiting Epoxide Reductase (vitamin K-->activated vitamin K)
-factors VIIa, IXa, Xa, XIa, XIIa
Drug that activates antithrombin?
Produced by S. aureus
-->can convert fibrinogen-->fibrin
tPA, Streptokinase, Urokinase:
All facilitate: plasminogen--> plasmin
(so, stimulate breakdown of clots!)
vWF receptor on platelets? Fibrinogen receptor on platelets?
-vWF --> GpIb
-Fibrinogen --> GpIIb/IIIa
***vWF and Fibrinogen are both inside platelets
ESR in pregnancy?
-sickle cell anemia
Spur cell (Acanthocyte)
Liver disease, Abetalipoproteinemia
-->irregularly spiked RBCs
-Anemia of chronic disease
-->have denatured RNA within RBCs
type of anemia caused by folate or B12 deficiency?
Macrocytic, Megaloblastic anemia
iron deficiency anemia + esophageal web + atrophic glossitis?
"crew cut" on skull x-ray?
Bone Marrow expansion --> see in beta-thal major
-Sickle cell disease
sideroblastic anemia = defect in heme synthesis (x-linked defect in ALA synthase gene)
-treat with B6 (Pyridoxine)
Lead poisoning symptoms: LEEAADDS
-Lead lines on gingivae (Burton's lines) and on epiphyses of long bones on x-ray
-Erythrocyte basophilic stippling
-Anemia (sideroblastic anemia)
-Drops (wrist and foot drop)
-Dimercaprol and EDTA = treatment
-Succimer = treatment for kids
treatment for lead poisoning (also EDTA)
=treatment for kids with lead poisoning ("SUCks to be a kid with lead poisoning")
Hypersegmented neutrophils + glossitis +
increased homocysteine and:
-normal methylmalonic acid?
-increased methylmalonic acid?
-if normal methylmalonic acid: Folate deficiency
-if increased methylmalonic acid: B12 deficiency
***both cause megaloblastic macrocytic anemia (so have impaired DNA synthesis and ineffective erythropoiesis = pancytopenia)
Megaloblastic anemia that's not correctable by vitamin B12 or Folate?
--> Orotic Aciduria
DNA repair defect --> get aplastic anemia
(NOT the same as Fanconi's syndrome = proximal tubule reabsorption defect in kidneys!)
Why anemia in kidney disease?
decreased EPO --> decreased hematopoiesis
-Normal cell morphology, but hypocellular bone marrow with fatty infiltration
Hemolytic anemia in a newborn?
Pyruvate kinase deficiency --> decreased ATP --> rigid RBCs
Glutamic acid-to-lysine mutation
*pts with HbSC (1 of each mutant gene) have milder disease than HbSS pts (full sickle cell)
older pt, unexplaned anemia:
must rule out colon cancer
reversible etiologies of sideroblastic anemia?
what vitamin should be supplemented in vegans/vegetarians?
B12 (may get deficiency --> megaloblastic anemia)
released by liver, binds ferroportin on intestinal mucosal cells and macrophages
-increased in cases of inflammation: increased hepcidin-->decrease release of iron from macrophages
*this is what happens in anemia of chronic disease: decreased iron, decreased TIBC, increased ferritin
decreased iron + decreased TIBC + increased ferritin
anemia of chronic disease (inflammation--> increased hepcidin --> decreased release of iron from macrophages
Cause of hereditary spherocytosis?
-extravascular intrinsic hemolytic normocytic anemia
-caused by defect in proteins interacting with RBC membrane skeleton and plasma membrane (ankyrin, band 3, protein 4.2, spectrin)
-premature removal of RBCs by spleen --> splenomegaly, aplastic crisis; eventually need splenectomy
Labs for PNH (paroxysmal nocturnal hemoglobinuria)?
increased urine hemosiderin
complication of PNH?
Treatment for sickle cell anemia?
-Hydroxyurea --> increases HbF
-Bone marrow transplant = ultimately
Coomb's positive anemia?
autoimmune hemolytic anemia (ie warm agglutinin or cold agglutinin anemias)
-RBCs damaged when passing through obstructed or narrowed vessel lumina
-see in: DIC, TTP-HUS, SLE, malignant HTN; prosthetic valves, aortic stenosis...
rate limiter of heme synthesis?
ALA synthase (delta-aminolevulinic acid synthase)
effect of heme on ALA synthase activity
low heme--> increased ALA synthase
lots of heme-->decreased ALA synthase
Rate limiter of heme synthesis
ALA synthase (requires B6!)
tea-colored urine + blistering cutaneous photosensitivity?
-Porphyria cutanea tarda (most common porphyria)
-d/t deficiency of Uroporphyrinogen Decarboxylase (needed for heme synthesis); get accumulation of Uroporphyrin (hence the tea-colored urine)
painful abdomen + red-wine colored urine + polyneuropathy + psych disturbances; precipitated by drugs:
Acute Intermittent Porphyria
-d/t deficiency of Porphobilinogen Deaminase (aka uroporphyrinogen-I-synthase)
-accumulate Porphobilinogen, ALA, and uroporphyrin (in urine)
Treatment for Acute Intermittent Porphyria?
-Glucose and Heme --> inhibit ALA synthase
Defect in factors I, II, V, VII, X -->?
increased PT (extrinsic pathway defect)
Defect in all factors EXCEPT VII and XIII?
Defects in platelet plug formation?
increased BT (bleeding time)
-labs (BT =bleeding time; PC=platelet count)
defect in Gp1b--> so vWF can't bind platelets for platelet plug formation
-defect in GpIIb/IIIa --> platelets can't link to eachother, so no platelet aggregation for platelet plug formation
-no effect on PC
ITP = Idiopathic Thrombocytopenic Purpura:
-defect:have anti-GpIIb/IIIa antibodies--> antibodies bind platelets --> complex gets consumed by splenic macrophages
-also have increased megakaryocytes (trying to make more platelets!)
TTP = Thrombotic Thrombocytopenic Purpura
deficiency of vWF metallprotease --> decreased degradation of vWF multimers: so: increased vWF multimers--> increased platelet aggregation and thrombosis--> decreased platelet survival
Affect of aspirin on BT/PC?
no effect on PC
All platelet disorders effects on BT/PC?
-all increase BT
-all decrease PC, except Glanzmann's
most common inherited bleeding disorder?
von Willebrand's disease
PC, BT, PT, PTT findings in von Willebrand's disease?
-increased or normal PTT (depends on severity)
-normal PC and PT
-treatment for von-Willebrand's disease?
DDAVP = Desmopressin (releases vWF stored in endothelium)
PC, BT, PT, PTT findings in DIC?
-other lab findings?
-also: schistocytes, increased D-dimers (fibrin split products), decreased fibrinogen, decreased factors V and VIII)
Causes of DIC?
"STOP Making New Thrombi"
Factor V Leiden
most common cause of inherited hypercoagulability
-production of mutant factor V, that can't be degraded by protein C
contains fibrinogen, factor, VIII, factor XIII
-used to treat coagulation factor deficiencies (if deficient in fibrinogen or factor VIII)
CD30+ and CD15+ cells of B-cell origin?
Most common Hodgkin's lymphoma? Prognosis?
-Excelent prognosis (b/c Lymphocytes>>>R-S cells)
-see collagen banding in lymph node
-mostly young adults
t(8;14) c-myc gene
Burkitt's lymphoma (non-Hodgkin lymphoma; neoplasm of mature B cells)
Most common adult non-Hodgkin lymphoma?
Diffuse large B-cell lymphoma
-->20% of cases are in kids though
t(11;14); affects cyclin D regulatory gene
Mantle Cell Lymphoma
-affects older males; poor prognosis
t(14;18) bcl-2 overexpression
-indolent course, but difficult to cure
-bcl-2 inhibits apoptosis (normally; it's a proto-oncogene)
Lymphoma caused by HTLV-1?
Adult T-cell lymphoma
-->adults present with cutaneous lesions
Mycosis fungoides/Sezary syndrome
-mature T-cell lymphoma; adults present with cutaneous lesions
-Ig light chains in urine (don't see on urinalysis though)
most common primary tumor arising within bone in elderly?
CRAB symptoms of Multiple Myeloma:
-Bone lytic lesions/Back pain
Down syndrome pts: increased risk of which cancers?
stains TRAP positive (tartrate-resistant acid phosphatase)
Hairy cell leukemia
-mature B-cell tumor in elderly; cells have filamentous, hairlike projections
Age group affected by ALL?
<15 years old
-most responsive to therapy
-may spread to CNS and testes
-t(12;21) = better prognosis
Hallmark of Acute Leukemias?
-lots of blast cells (>20% blasts)
-rapid onset and progression
AML = t(15;17)
-responds to vitamin A
Which leukemia can be treated with Vitamin A?
-30-60 yrs old
-more mature cells (<5% blasts); insidious onset
-resonds to imatinib = bcr-abl tyrosine kinase inhibitor
treatment for CML
-inhibits bcr-abl tyrosine kinase
-->fibrosis of bone marrow (
-->bone marrow is crying because it's fibrosed :(
JAK2 mutations: positive/negative?
-positive in polycythemia vera, essential thrombocytosis, myelofibrosis
-negative in CML
-only positive in CML
thombolytics mechanism? (streptokinase, urokinase, tPA, APSAC)
aid conversion of plasminogen-->plasmin, which cleaves thrombin and fibrin clots
Affect of aspirin on BT, PT, PTT
-no effect on PT, PTT
-Neutropenia (should monitor CBC during first few months of trtmt)
(Ticlopidine = ADP-receptor blocker, so inhibits platelt aggregation; like Clopidogrel)
Drug side effect = tinnitus?