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250 Cards in this Set

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Erythropoietin
synthesized in peritubular capillaries
Reticulocyte count
measure of effective erythropoiesis; correct for degree of anemia
Extramedullary hematopoiesis
hematopoiesis outside bone marrow (e.g., spleen)
Newborn physiologic anemia
drop in Hb due to replacement of HbF RBCs with HbA
Pregnancy Heme
Hb and Hct decreased; greater increase in plasma volume than RBC mass
Anemia
normal 02 saturation and arterial P02
MCV
average volume of RBCs; useful for anemia classification
MCHC definition
average Hb concentration in RBCs
MCHC specifics
dec in microcytic anemias; inc in spherocytosis
Thalassemias
dec MCV, inc RBC count
RDW
RBC size variation; inc iron deficiency; normal in other microcytic anemias
Mature RBC
anaerobic glycolysis; no mitochondria or HLA antigens
Total iron binding capacity
inc iron deficiency; dec anemia chronic disease, sideroblastic anemia
% Saturation
dec iron deficiency, anemia chronic disease; inc sideroblastic anemia
Serum ferritin
dec in iron deficiency; inc anemia chronic disease, sideroblastic anemia, normal thalassemia
Microcytic anemias
iron deficiency MC, anemic chronic disease, thalassemia, sideroblastic anemia
Iron deficiency child
MCC Meckel's diverticulum
Iron deficiency woman < 50
MCC menorrhagia
Iron deficiency man < 50
MCC peptic ulcer disease
Iron deficiency men/woman > 50
MCC colon cancer
Stages iron deficiency
dec ferritin; de Fe and % saturation, inc TIBC; normocytic then microcytic anemia
Anemia chronic disease
MC anemia in malignancy and alcoholics
a-Thalassemia trait
AR; two a-globin gene deletions; normal Hb electrophoresis
HbH disease
three a-globin gene deletions; hemolytic anemia; four beta-globin chains
Hb Bart's disease
four a-globin gene deletions; four gamma-globin chains
beta-Thalassemia minor
AR; DNA splicing defect; T HbA2 and F; .L HbA
beta-Thalassemia major
nonsense mutation with stop codon; hemolytic anemia; TTHbF, T HbA2
Sideroblastic anemia
defect in mitochondrial heme synthesis producing ringed sideroblasts
Causes sideroblastic anemia
alcohol, pyridoxine deficiency (isoniazid Rx of TB), Pb poisoning
Pb poisoning
inhibition ferrochelatase, S-aminolevulinic acid dehydrase, ribonuclease
S/S Pb poisoning in children
growth retardation; Pb in epiphyses; abdominal colic; encephalopathy
S/S Pb poisoning in adult
peripheral neuropathy; proximal renal tubule damage (Fanconi's syndrome)
Labs in Pb poisoning
coarse basophilic stippling RBCs; dec MCV; inc blood Pb; inc S-aminolevulinic acid
Vitamin B12:
animal products; requires intrinsic factor for reabsorption in terminal ileum
Vitamin B12 biochemical function
transfers methyl group to homocysteine
R factor
binds with B12 in mouth, removed by pancreatic enzymes in small intestine
Vitamin B12 involvement in metabolism
involved in propionate metabolism; end-product succinyl CoA
Causes of B12 deficiency
vegan, pernisicious anemia MC, fish tapeworm, pancreatitis, bacterial overgrowth, Crohn's disease
Pernicious Anemia
autoimmune destruction of parietal cells; chronic gastritis body/fundus; achlorhydria; inc gastrin
Causes of folate deficiency
alcohol MCC, poor diet, drugs, malabsorption, pregnancy, goat milk
Drugs and folate deficiency
alcohol, OC, phenytoin, methotrexate, trimethoprim, 5-fluorouracil
Intestinal conjugase in folate metabolism
inhibited by phenytoin
Jejunal uptake of monoglutamate form of folate
inhibited by alcohol and OC
Dihydrofolate reductase
inhibited by methotrexate, trimethoprim
Thymidylate synthetase
Thymidylate synthetase
Folate deficiency
MCC of increased serum homocysteine
Labs in B12/folate deficiency
pancytopenia; hypersegmented neutrophils; inc homocysteine
Lab findings unique to B12 deficiency
inc gastrin (PA), inc methylmalonic acid
B12 reabsorbed/absorbed after administration of intrinsic factor
pernicious anemia
B12 reabsorbed/absorbed after administration of antibiotics
bacterial overgrowth
B12 reabsorbed/absorbed after administration of pancreatic extract
chronic pancreatitis
Acute blood loss
initially normal Hb and Het; 0.9% saline uncovers RBC deficit
Aplastic anemia caused by:
drugs (e.g., phenylbutazone); infection (e.g., parvovirus); benzene
Lab findings in aplastic anemia
pancytopenia; hypocellular bone marrow
Anemia in renal disease
normocytic; decreased EPO
Extravascular hemolysis
macrophage phagocytosis of RBCs; inc unconjugated bilirubin and urine UBG
Intravascular hemolysis
dec serum haptoglobin; hemoglobinuria; hemosiderinuria
Congenital spherocytosis
AD; defect in spectrin; extravascular hemolysis; splenomegaly
Blood findings in spherocytosis
normocytic anemia; dense RBCs, inc MCHC, inc osmotic fragility
Paroxysmal nocturnal hemoglobinuria
missing decay accelerating factor; complement destruction RBCs, neutrophils, platelets
S/S Paroxysmal nocturnal hemoglobinuria
pancytopenia; hemoglobinuria; positive sugar water test and acidified serum test
HbSS
AR; missense mutation (valine for glutamic acid 6th positive (3-globin chain)
Causes of sickling
inc deoxyhemoglobin (hypoxemia, acidosis); HbS > 60%
HbF
inhibits sickling; hydroxyurea inc HbF
Pathophysiology HbSS
vasoocclusive crises, hemolytic anemia (extravascular)
HbSS in children
dactylitis (6-9 mths); Streptococcus pneumoniae sepsis (dysfunctional spleen)
HbSS and osteomyelitis
Salmonella paratyphi
HbSS complications
aplastic crisis (parvovirus), acute chest syndrome, autosplenectomy, calcium bilirubinate gallstones, priapism, aseptic necrosis
HbAS
microhematuria from sickling in renal medulla; renal papillary necrosis
Hb electrophoresis
HbAS- HbA 55-60%, HbS 40-45%; HbSS- HbS 90-95%, HbF 5-10%
Blood findings in HbSS
sickle cells; target cells; Howell-Jolly bodies (nuclear remnants)
G6PD deficiency
XR; oxidant damage (peroxide) to Hb (e.g., primaquine; dapsone; fava beans)
Blood findings G6PD deficiency
Heinz bodies (denatured Hb; special stain); bite cells
Pyruvate kinase deficiency
dec ATP; RBCs dehydrated; inc 2,3-BPG (right-shifted OBC)
Warm type Autoimmune hemolytic anemia
IgG; extravascular hemolysis; e.g, SLE, drugs
Cold type Autoimmune hemolytic anemia
IgM intravascular hemolysis; e.g., CLL, Mycoplasma
Penicillin and hemolysis
IgG antibody against penicillin attached to RBC (type II hypersensitivity)
Methyldopa and hemolysis
drug alters Rh antigens; IgG antibody against Rh antigens (type II hypersensitivity)
Quinidine and hemolysis
drug-IgM IC; intravascular hemolysis; type III hypersensitivity
Lab findings in autoimmune hemolytic anemia
positive direct Coombs'; spherocytes
Micro-macroangiopathic hemolysis
mechanical damage causing intravascular hemolysis
Causes of micro/macro hemolysis
aortic stenosis (MCC), DIC, TTP, HUS
Peripheral blood findings in micro/macro hemolysis
schistocytes; iron deficiency from hemoglobinuria
Malaria and hemolysis
intravascular hemolysis correlates with fever; falciparum- ring forms and gametocytes
Leukemoid reaction
exaggerated WBC response to infection; usually due to infection
Leukoerythroblastic reaction
marrow infiltrative disease peripheralizes myeloblasts/nucleated RBCs
Causes of leukoerythroblastic reaction
bone metastasis MCC, myelofibrosis
Eosinophilia
type I hypersensitivity (e.g., penicillin reaction); invasive helminthic infection
Helminths not producing eosinophilia:
pinworms, adult worms in ascariasis
Atypical lymphocytes
mononucleosis; CMV; toxoplasmosis; viral hepatitis; phenytoin
Mononucleosis
due to EBV; EBV attaches to CD21 receptors on B cells
Clinical findings in mono
exudative tonsillitis, generalized lymphadenopathy, hepatosplenomegaly
Lab findings in mono
atypical lymphocytosis; IgM heterophile antibodies against horse RBCs
Lymphopenia
T cell deficiencies (HIV); combined B/T deficiency (adenine deaminase deficiency)
Lymphocytosis
viral infections, whooping cough
Corticosteroids and WBC
lymphopenia, eosinopenia, neutrophilia
Chronic Myeloproliferative disorder
neoplastic stem cell disorder; splenomegaly; marrow fibrosis; risk for leukemia
Examples of myeloproliferative disorder
polycythemia vera, myelofibrosis and myeloid metaplasia
Relative polycythemia
plasma volume; inc RBC count; normal RBC mass
Absolute polycythemia
inc RBC count and RBC mass
Appropriate polycythemia
hypoxic stimulus for EPO to generate RBCs
Causes of appropriate absolute polycythemia
lung disease, cyanotic heart disease, high altitude
Appropriate absolute polycythemia
normal plasma volume; inc RBC mass; dec Sa02; inc EPO
Inappropriate absolute polycythemia
no hypoxic stimulus for EPO
Causes of inappropriate polycythemia
ectopic secretion EPO, polycythemia vera
Polycythemia vera
inc plasma volume and RBC mass; normal Sa02; dec EPO
Ectopic EPO (renal cell carcinoma)
normal plasma volume; inc RBC mass; normal Sa02; inc EPO
Myelofibrosis myeloid metaplasia
marrow fibrosis; extramedullary hematopoiesis; splenomegaly
Lab findings in myelofibrosis
tear drop RBCs; dry bone marrow aspirate (marrow fibrosis)
Essential thrombocythemia
MPD with increase in abnormal appearing platelets
Myelodysplastic syndrome
severe anemia in elderly; 30% develop leukemia; ringed sideroblasts
Benzene causes
aplastic anemia; acute leukemia
Leukemia by age
ALL: newborn-14; AML: 15-60; CML: 40-60; CLL: > 60
Acute vs chronic leukemia
acute: blasts > 30% in bone marrow; chronic: blasts < 10% in bone marrow
AML
Auer rods in myeloblasts
Acute promyelocytic leukemia
t(15;17); defect in retinoic acid; Rx retinoic acid (T maturation); DIC
Acute monocytic leukemia
gum infiltration
CML
t(9;22) of ABL POC; Philadelphia chromosome 22; dec leukocyte alkaline phosphatase score
ALL
early pre-B (80%); CALLA (CD10) and TdT positive; CNS and testicle involvement
ALL
t(12;21) offers good prognosis
CLL
B cell neoplasm; dec gamma-globulins; MCC generalized lymphadenopathy patients > 60-yrs-old
Adult T cell leukemia
HTLV-1; CD4 T cells; skin infiltration; lytic bone lesions with hypercalcemia
Hairy cell leukemia
positive TRAP stain; splenomegaly; Rx with purine nucleosides
Nodal sites of cells
germinal follicles: B cells; paracortex: T cells; sinuses: histiocytes
Testicular cancer
metastasizes to para-aortic nodes
Stomach cancer
metastasizes to left supraclavicular nodes (Virchow node)
Phenytoin and peripheral smear
atypical lymphocytosis
Cat scratch disease
Bartonella henselae; granulomatous microabscesses
Follicular B-cell lymphoma
t(14;18); overexpression of BCL-2 anti-apoptosis gene
Burkitt lymphoma
t(8; 14); EBV association; common childhood NHL; "starry sky" appearance
Extranodal lymphomas: risk factors
H. pylori (stomach); Sjogren's syndrome
Mycosis fungoides
CD4 T neoplasm; skin lesions with Pautrier's microabscesses
Sezary syndrome
leukemic phase of mycosis fungoides
Polyclonal gammopathy
sign of chronic inflammation
Monoclonal gammopathy
M component (spike); sign of plasma cell disorder
Confirmatory tests for leukemia
serum and urine immunoelectrophoresis; bone marrow aspirate
Bence Jones protein
light chains in urine; predictive of a malignant plasma cell disorder
Multiple myeloma
M spike; lytic bone lesions; pathologic fractures; hypercalcemia; renal failure
MGUS (monoclonal gammapathy of undetermined significance)
MC monoclonal gammopathy; may progress to myeloma
Findings in MGUS (monoclonal gammapathy of undetermined significance)
elderly patient; no BJ protein; no malignant plasma cells
Waldenstrom's macroglobulinemia
lymphoplasmacytic lymphoma; IgM M spike; hyperviscosity
Hodgkin's lymphoma
neoplastic component Reed Sternberg (RS) cell; CD 15 CD30 positive
Lymphocyte predominant Hodgkin's
infrequent classic RS cells
Nodular sclerosing Hodgkin's
female dominant supraclavicular nodes + anterior mediastinal nodes
Mixed cellularity Hodgkin's
male dominant; numerous RS cells; EBV association
Hodgkin's prognosis
stage of disease and type of Hodgkins most important factors
Alkylating agents in Rx of Hodgkins
inc risk for second malignancies (leukemia; NHL)
Langerhan's histiocytes
CD1 positive; Birbeck granules
Letterer-Siwe disease
malignant histiocytosis < 2-yrs-old; diffuse eczematous rash; organ involvement
Hand-Christian-Christian disease
malignant; lytic skull lesions, diabetes insipidus, exophthalmos
Eosinophilic granuloma
benign histiocytosis; lytic bone lesions with pathologic fractures
Mast cells
release histamine (pruritus; swelling); metachromatic granules positive with toluidine blue
Urticaria pigmentosum
localized mastocytosis; skin lesions swell and itch with scratching
Amyloid
twisted beta-sheet; apple green birefringence
Primary amyloidosis
AL amyloid derived from light chains; plasma cell disorders
Secondary amyloidosis
AA amyloid derived from serum-associated amyloid; chronic infections
Alzheimer's disease
amyloid precursor protein gene product chromosome 21; amyloid-beta
Gaucher's disease
macrophages have fibrillary appearance; deficiency glucocerebrosidase
Niemann Pick's disease
macrophages have soap bubble appearance; deficiency sphingomyelinase
Hypersplenism
splenomegaly; peripheral blood cytopenias; portal hypertension MCC
Splenic dysfunction
Howell Jolly bodies; susceptible to Streptococcus pneumoniae sepsis
Anticoagulants
tissue plasminogen activator, heparin, PGI2, ATIII, protein C/S
Heparin MOA
enhances ATIII activity (neutralizes all factors except V, VIII, fibrinogen)
Protein C/S
neutralize V and VIII
Procoagulants
coagulation factors, thromboxane A2 (platelet aggregation, vasoconstrictor)
Protein C and S
inactivate factors V and VIII; enhance fibrinolysis
Von Willebrand factor
complexes with factor VIII to enhance VIII:C activity; platelet adhesion
Platelets
receptors for von Willebrand factor and fibrinogen; synthesize thromboxane A2
GpIb
platelet receptor for von Willebrands factor
GpIIb:IIIa
platelet receptor for fibrinogen
Extrinsic system factor
VII
Intrinsic system factors
XII, XI, IX, VIII
Final common pathway factors
X, V, prothrombin (II), fibrinogen (I)
Factor XIII
cross-links insoluble fibrin; strengthens fibrin clots
Vitamin K-dependent factors
prothrombin, VII, IX, X, protein C and S
Factors consumed in a clot
fibrinogen, prothrombin, V, VIII; fluid is called serum
Plasmin
cleaves fibrinogen and insoluble fibrin into degradation products
Bleeding time
Bleeding time
Aspirin and bleeding
MCC of a prolonged bleeding time
Tests for vWF
ristocetin cofactor assay; vWF antigen assay; agar electrophoresis
PT (prothrombin time)
evaluates extrinsic pathway to fibrin clot
PTT (partial thromboplastin time)
evaluates intrinsic pathway to stable fibrin clot
Fibrinolysis tests
fibrin(ogen) degradation products; D-dimers (cross-linked insoluble fibrin)
S/S platelet dysfunction
cannot form temporary plug; epistaxis; petechiae; bleeding from scratches
Idiopathic thrombocytopenic purpura (ITP)
children; antibodies against GpIIb:IIla; no splenomegaly
Chronic autoimmune thrombocytopenic purpura
SLE; antibodies against GpIIb:IIIa receptors
Heparin and platelets
thrombocytopenia due to IgG antibody against heparin attached to PF4 on platelets
PF4
heparin neutralizing factor
HIV hematologic complication
thrombocytopenia MC hematologic abnormality; similar to ITP
TTP
platelet thrombi develop in areas of endothelial damage in small vessels; consumption of platelets
S/S of TTP
fever, thrombocytopenia, renal failure, hemolytic anemia with schistocytes, CNS deficits
Lab findings in TTP
thrombocytopenia, prolonged bleeding time, normal PT and PTT
HUS
similar to TTP; endothelial injury from Shiga-like toxin of 0157:H7 E. coli in undercooked beef
S/S of factor deficiency
no stable fibrin clot- late rebleeding; menorrhagia; GI bleeding; hemarthroses
Hemophilia A
XR; hemarthroses; prolonged PTT, dec factor VIII activity, normal VIII antigen
Von Willebrand's disease
AD; platelet adhesion defect + factor VIII deficiency
Lab findings in VWD
dec vWF, VIII antigen, and VIII:C; prolonged bleeding time
Desmopressin acetate
Rx of choice for mild von Willebrand's disease and hemophilia A
Circulating anticoagulants
antibodies destroy coagulation factors
Lab finding in circulating anticoagulant
prolonged PT and/or PTT corrected with mixing studies
Vitamin K deficiency
dec epoxide reductase activity (dec function vitamin K); hemorrhagic diathesis; inc PT
Causes of vitamin K deficiency
antibiotics MC, newborn, malabsorption, warfarin
DIC
activation coagulation system from release of tissue thromboplastin and/or endothelial cell damage
DIC
consumption coagulation factors by fibrin clots; patient also anticoagulated
causes of DIC
septic shock MCC, rattlesnake bite, massive trauma, amniotic fluid
S/S of DIC
bleeding from all scratches, holes, needle sites
Lab findings in DIC
thrombocytopenia, inc PT and PTT, D-dimers (best test), anemia
Antiphospholipid antibodies
lupus anticoagulant and anticardiolipin antibodies; vessel thrombosis
Warfarin
inhibits epoxide reductase; PT best test but PTT also prolonged
Warfarin
full anticoagulation in 3 days when gamma-carboxylated prothrombin disappears
Warfarin
ingredient in rat poison; danger to children in households with grandparents on warfarin
Rx for warfarin overanticoagulation
intramuscular vitamin K (6-8 hrs), fresh frozen plasma (immediate)
Heparin
enhances ATIII; PTT best test but PT also prolonged
oral contraceptives
estrogen inc coagulation factor synthesis and 4- ATIII; predisposes to thrombosis
Factor V Leiden
MC hereditary thrombosis; resistant to degradation by protein C/S
ATIII deficiency
no prolongation of PTT with administration of heparin
Hemorrhagic skin necrosis
post-warfarin therapy in patient with heterozygote protein C deficiency
M cells
specialized cells that transfer foreign antigens to lymphocytes in Peyer's patches
Blood group O
some patient have anti-AB-IgG antibodies; increased incidence duodenal ulcers
Blood group A
increased incidence of gastric carcinoma
newborns and blood group
do not have natural blood group antibodies at birth (e.g., anti-A-IgM)
Elderly and blod groups
may lose natural blood group antibodies; no hemolytic reaction to mismatched blood
Rh antigens
inherited in autosomal codominant fashion; Rh antigens include D, C, c, E, e
Atypical antibodies
antibodies against Rh or non-Rh blood group antigens (e.g., anti-D)
Duffy antigen
receptor for Plasmodium vivax; blacks often lack
Duffy antigen
Antibody screen
indirect Coomb's test; detects atypical antibodies in serum
Cytomegalovirus
MC infection transmitted by blood transfusion; MC antibody
MCC of post-transfusion hepatitis
Hepatitis C
Major crossmatch
patient serum reacted against donor RBCs; does not guarantee RBC survival
Universal donor
blood group O; no antigens on the surface of RBCs
Universal recipient
blood group AB; no natural blood group antibodies in serum
Packed RBC transfusion
raises Hb by 1 gm/dL and Hct by 3%
Cryoprecipitate
fibrinogen and factor VIII
Fresh frozen plasma
replacement for multiple factor deficiencies (e.g., cirrhosis, DIC)
Allergic transfusion reaction
type I IgE-mediated hypersensitivity reaction
Febrile transfusion reaction
recipient anti-HLA antibodies react against donor leukocytes
Intravascular HTR
transfusion of ABO incompatible blood (e.g., A person receives B blood)
Extravascular HTR
antibody attaches to donor RBCs; macrophage phagocytosis and hemolysis
Positive direct Coomb's test
present in both types of hemolytic transfusion reactions
S/S of hemolytic transfusion reaction
jaundice, no increase in Hb, hemoglobinuria
ABO Hemolytic disease of the newborn
mother O and baby A or B; transplacental passage of maternal anti-AB-IgG
ABO Hemolytic disease of the newborn
positive direct Coomb's test; spherocytes; MCC unconjugated hyperbilirubinemia first 24-hrs
Rh Hemolytic disease of the newborn
mother Rh (D antigen) negative and fetus Rh (D antigen) positive
Rh Hemolytic disease of the newborn
no hemolysis in first Rh incompatible pregnancy
Rh Hemolytic disease of the newborn
maternal anti-D crosses placenta; potential for hydrops fetalis; high risk for kernicterus
Rh immune globulin
anti-D; coats D antigen site on fetal RBCs in maternal circulation
Rh Hemolytic disease of the newborn lab
positive direct Coomb's; severe anemia and hyperbilirubinemia
ABO Hemolytic disease of the newborn
protects mother from Rh sensitization (development of anti-D antibodies)
O Rh negative mother with A Rh positive baby
A+ cells destroyed by mothers anti A-IgM
Blue fluorescent light
converts unconjugated bilirubin in skin into harmless water soluble dipyrrole