Goljan 2 - Sheet1 Flashcards
CLL
B cell neoplasm; ? ?-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
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
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; Òstarry skyÓ appearance
Extra nodal lymphomas
risk factors H. pylori (stomach); SjogrenÕs syndrome
Mycosis fungoides
CD4 T cell neoplasm; skin lesions with PautrierÕs 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
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
MC monoclonal gammopathy; may progress to myeloma
Findings in MGUS
elderly patient; no BJ protein; no malignant plasma cells
WaldenstromÕs macroglobulinemia
lymphoplasmacytic lymphoma; IgM M spike; hyperviscosity
HodgkinÕs lymphoma
neoplastic component, Reed Stemberg (RS) cell; CD15 CD30 positive
Lymphocyte predominant HodgkinÕs
infrequent classic RS cells
Nodular sclerosing HodgkinÕs
female dominant; supraclavicular nodes + anterior mediastinal nodes
Mixed cellularity HodgkinÕs
male dominant; numerous RS cells; EBV association
HodgkinÕs prognosis
stage of disease and type of HodgkinÕs most important factors
Alkylating agents in Rx of Hodgkins
? risk for second malignancies (leukemia; NHL)
LangerhanÕs 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 ?-sheet; apple green birefringence with Congo red
Primary amyloidosis
AL amyloid derived from light chains; plasma cell disorders
Secondary amyloidosis
AA amyloid derived from serum-associated amyloid; chronic infections
AlzheimerÕs disease
amyloid precursor protein gene product chromosome 21; amyloid-?
GaucherÕs disease
macrophages have fibrillary appearance; deficiency glucocerebrosidase
Niemann PickÕs 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
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 Willebrand 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
evaluates platelet function (adhesion, release reaction, aggregation)
Aspirin
MCC of a prolonged bleeding time
Tests for vWF
ristocetin cofactor assay; vWF antigen assay; agar electrophoresis
PT
evaluates extrinsic pathway to fibrin clot
PTT
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:IIIa; no splenomegaly
Chronic autoimmune thrombocytopenic purpura
SLE; antibodies against GpIIb:IIIa receptors
Heparin
thrombocytopenia due to IgG antibody against heparin attached to PF4 on platelets
PF4
heparin neutralizing factor
HIV
thrombocytopenia MC hematologic abnormality; similar to ITP
TTP
platelet thrombi develop in areas of endothelial damage in small vessels; consumption of platelets
S/S
fever, thrombocytopenia, renal failure, hemolytic anemia with schistocytes, CNS deficits
Lab findings 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 factor deficiency
no stable fibrin clot-late rebleeding; menorrhagia; GI bleeding; hemarthroses
Hemophilia A
XR; hemarthroses; prolonged PTT, ? factor VIII activity, normal VIII antigen
von WillebrandÕs disease
AD; platelet adhesion defect + factor VIII deficiency
Lab findings in VWD
? vWF, VIII antigen, and VIII:C; prolonged bleeding time
Desmopressin acetate
Rx of choice for mild von WillebrandÕs 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
? epoxide reductase activity (? function vitamin K); hemorrhagic diathesis; ? PT
Causes 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
septic shock MCC, rattlesnake bite, massive trauma, amniotic fluid
S/S
bleeding from all scratches, holes, needle sites
Lab findings DIC
thrombocytopenia, ? 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 -carboxylated prothrombin disappears
Warfarin
ingredient in rat poison; danger to children in households with grandparents on warfarin
Rx warfarin over anticoagulation
intramuscular vitamin K (6-8 hrs), fresh frozen plasma (immediate)
Heparin
enhances ATIII; PTT best test but PT also prolonged
OC
estrogen ? coagulation factor synthesis and 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 PeyerÕs patches
Blood group O
some patients have anti-AB-IgG antibodies; increased incidence duodenal ulcers
Blood group A
increased incidence of gastric carcinoma
Newborns
do not have natural blood group antibodies at birth (e.g., anti-A-lgM)
Elderly
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 CoombÕs test; detects atypical antibodies in serum
Cytomegalovirus
MC infection transmitted by blood transfusion; MC antibody
Hepatitis C
MCC of post-transfusion hepatitis
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 CoombÕs test
present in both types of hemolytic transfusion reactions
S/S
jaundice, no increase in Hb, hemoglobinuria
ABO HDN
mother O and baby A or B; transplacental passage of maternal anti-AB-IgG
ABO HDN
positive direct CoombÕs test; spherocytes; MCC unconjugated hyperbilirubinemia first 24 hrs
Rh HDN
mother Rh (D antigen) negative and fetus Rh (O antigen) positive
Rh HDN
no hemolysis in first Rh incompatible pregnancy
Rh HDN
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 HDN lab
positive direct CoombÕs; severe anemia and hyperbilirubinemia
ABO HDN
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-lgM
Blue fluorescent light
converts unconjugated bilirubin in skin into harmless water soluble dipyrrole
MV auscultation
apex
TV auscultation
left parasternal border
AV auscultation
right 2nd intercostal space
PV auscultation
left 2nd intercostal space
S1
closure MV and TV
S2
closure AV and PV
Inspiration
split in A2 and P2; due to increased blood in right side of heart
S3
abnormal; due to blood entering volume overloaded ventricle in early diastole
Causes S3
valve regurgitation; congestive heart failure
S4
abnormal; due to blood entering non-compliant ventricle with atrial contraction in late diastole
Causes S4
volume overloaded ventricle, hypertrophy
Murmurs
stretching valve ring or damage to valve
Inspiration
increases right sided abnormal heart sounds and murmurs
Expiration
increases left sided abnormal heart sounds and murmurs
Stenosis murmurs
problem in opening valve
Regurgitation murmurs
problem in closing valve
Valves opening in systole
AV and PV
Valves opening in diastole
MV and TV
Valves closing in systole
MV and TV
Valves closing in diastole
AV and PV
LDL
primary vehicle for carrying cholesterol
VLDL
primary vehicle for carrying liver-synthesized triglyceride
Familial hypercholesterolemia (type II)
AD; deficiency of LDL receptors; ? LDL
Type III hyperlipoproteinemia
deficiency apo E; ? remnants (chylomicron, intermediate density)
Type IV hyperlipoproteinemia
? VLDL; alcoholics
Apo B deficiency
deficiency apo B48 (chylomicrons) and B100 (VLDL); ? CH and TG
Clinical findings in apo B deficiency
malabsorption; hemolytic anemia
Atherosclerosis
reaction to injury of endothelial cells
Risk factors
smoking, ? LDL, ? homocysteine, Chlamydia pneumoniae infection
Cells involved
platelets, macrophages, smooth muscle cells, T cells with cytokine release
Fibrous plaque
pathognomonic lesion of atherosclerosis
C-reactive protein
marker of an inflammatory atheromatous plaque
Inflammatory atheromatous plaque
predisposes to platelet thrombosis
Increased plasma homocysteine
? vessel thrombosis; folate (MC)/vitamin B12 deficiency
Hyaline arteriolosclerosis
small vessel disease of DM and hypertension; excess protein in vessel wall
Mechanisms hyaline arteriolosclerosis in DM
non-enzymatic glycosylation
Non-enzymatic glycosylation
glucose attaches to amino acids in BM; causes ? permeability to protein
Mechanisms hyaline arteriolosclerosis in hypertension
pressure pushes proteins into vessel wall
Abdominal aortic aneurysm rupture
due to atherosclerosis; flank pain, hypotension, pulsatile mass
Syphilitic aneurysm
vasculitis of vasa vasorum of aortic arch; aortic regurgitation
Aortic dissection
due to hypertension and collagen tissue disorders (e.g., Marfan)
Cystic medial degeneration
elastic tissue degeneration creates spaces filled with mucopolysaccharides
Intimal tear in aorta
due to wall stress from hypertension and structural weakness
Types of dissection
proximal (MC); distal or combination of both
S/S proximal aortic dissection
chest pain radiating to back, lack of pulse; cardiac tamponade MC COD
MarfanÕs
AD; fibrillin defect; aortic regurgitation/dissection; lens dislocation; MVP with sudden death
MC COD MarfanÕs and Ehlers Danlos
aortic dissection
Phlebothrombosis
stasis of blood flow; deep veins below knee MC site
Pulmonary thromboembolism
emboli originate from femoral veins
Superficial migratory thrombophlebitis
sign of carcinoma of head of pancreas
Thoracic outlet syndrome
absent radial pulse with positional change
TurnerÕs syndrome
lymphedema hands/feet in newborn; preductal coarctation
Spider telangiectasia
arteriovenous fistula; due to hyperestrinism (cirrhosis, pregnancy)
Capillary hemangioma in newborn
regress with age; do not surgically remove
KaposiÕs sarcoma
HHV-8; vascular malignancy; MC cancer in AIDS
Bacillary angiomatosis
Bartonella henselae; vascular infection in AIDS
Small vessel vasculitis
palpable purpura; e.g., Henoch Schonlein purpura
Muscular artery vasculitis
vessel thrombosis with infarction; e.g., classical polyarteritis nodosa
Elastic artery vasculitis
absent pulse, stroke
TakayasuÕs arteritis
pulseless disease; young Asian woman
Giant cell arteritis
temporal artery granulomatous vasculitis; ipsilateral blindness (ophthalmic artery)
Classical polyarteritis nodosa
muscular artery vasculitis with vessel thrombosis infarction
Path findings
vessel inflammation at different stages; aneurysms from vessel weakness
S/S
infarctions in kidneys, skin, GI tract, heart; HBsAg in 30%
Diagnosis
angiography identifies aneurysms and thrombosis
KawasakiÕs disease
coronary artery vasculitis/thrombosis/aneurysms in children
S/S
chest pain; desquamating rash; swelling hands/feet; cervical lymphadenopathy
Rx
IV ?-globulin
BuergerÕs disease (thromboangiitis obliterans)
smokerÕs digital vasculitis; digital infarction