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Heme II > other red cell disorders > Flashcards

other red cell disorders Flashcards

1
Q

relative polycythemias

A

dehydration (water deprivation, vomiting, diarrhea, diuretics)
stress polycythemia/baisbock syndrome- HTN, obese, anxious, unknown etiology

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2
Q

primary absolute polycythemias

A

intrinsic abnormality of hematopoietic precursors
PCV (MPD) most common
rare inherited erythropoietin receptor mutations

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3
Q

secondary absolute polycythemias

A

red cell progenitors respondning to increased erythropoietin

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4
Q

compensatory causes of secondary absolute polycythemias

A

lung disease
high altitude
cyanotic heart disease

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5
Q

paraneoplastic causes of secondary absolute polycythemias

A

erythropoietin secreting tumors: renal cell carcinoma, hepatocellular carcinoma, cerebellar hemagioblastoma

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6
Q

inherited defects causing secondary absolute polycythemias

A

VHL

prolyl hydroxylase mutations

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7
Q

general causes of hemorrhagic diatheses

A

increased fragility of vessels
platelet deficiency or dysfunction
drrangement of coagulation

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8
Q

PT

A

prothrombin time
extrinsic and common coagluation pathways
factor V, VII, X

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9
Q

PTT

A

partial thromboplastin time
intrinsic and common pathwyas
V, VIII, IX, X, XI, XII, prothrombin, fibrinogen, or interfering Abs to phospholipis

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10
Q

bleeding disorders caused by vessel wall abnormalities

A

relatively common, not usually serious bleeding
small hemorrhages in skin or mucous membranes
rarely into joints, mm, subperiosteal, GI, nosebleeds

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11
Q

causes of bleeding disorders caused by vessel wall abnormalities

A 
infections
drug rxns
connective tissue disease
hencoh-schonelein purpura
hereditary hemorrhagic telangiectasia- most serious
perivascular amyloidosis
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12
Q

infections causing bleeding disorders caused by vessel wall abnormalities

A 
petechial and purpuric hemorrhages of chest and back
mengiococcemia
septicemia
infective endocarditis
ricketssial diseases
vasculitis, DIC
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13
Q

drug rxns bleeding disorders caused by vessel wall abnormalities

A

petchiae and purpura w/o thrombocytopenia

depostion of ICs in vessel walls -? leukcocytoclastic vasculitis

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14
Q

CT disorders bleeding disorders caused by vessel wall abnormalities

A

scurvey
ehlers-danos syndrome
cushings
microvascular bleeding

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15
Q

henoch-schonelein purpura

A

systemic immune disorder of unknown cause
purpuric rash, colicky abdominal pain, polyarthralgia, acute glomerulonephritis
deposition of IgA in glomeruli and elsewhere

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16
Q

hereditary hemorrhagic telangiectasia

A

aka weber-osler-rendu syndrome
autosomal dominant
mutations in proteins which regulate TGFbeta signaling
dilated torturous blood vessels w/thin walls
bleeding most common from nose
this is most serious of vessel wall bleeding disorders

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17
Q

thrombocytopenia

A

<20,000 spontaneous bleeding

normal PT and PTT

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18
Q

causes of thrombocytopenia

A

decreased platelet production
decreased platelet survival
sequestration
dilution- massive transfusion

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19
Q

decreased platelet survival

A

immune thrombocytopenia- destruction due to Abs on platelets (IgG from mom)
DIC, and thrombocytic microangiopathies
mechanical injuries

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20
Q

chronic ITP

A

Ab mediated destruction of platelets

secondary due to SLE, HIV, B-cell neoplasms (CLL)

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21
Q

chronic ITP pathogenesis

A

Abs most often directed against platelet membrane glycoproteins IIb-IIIa or Ib-IX
almost always IgG
usually marked improvement with splenectomy b/c less opsonization and less Ab production
Abs may also bind and damage megakaryocytes

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22
Q

ITP morphology

A

principal changes found in spleen, bone marrow, and blood, but not specific
spleen normal size w/prominent reactive germinal centers
marrow reveals a modestly increased number of megakaryocytes
peripheral blood often reveals abnormally large platelets

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23
Q

ITP clinical

A

chronic ITP occurs most commonly in adult women <40
characterized by bleeding into skin and mucosal surfaces
petechiae, especially where capillary pressure is high
may manifest first w/melena, hematuria, or excessive menstruation
brain hemorrhages serious and fatal
spleneomegaly and lymphadenopathy are uncommon
PT and PTT normal
Dx of elxusion
almost all respond to corticosteroids
splenectomy work in 2/3

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24
Q

Acute ITP

A

mainly disease of childhood, equal M:F
Abs against platlets
1-2 wks post viral illness
self-limiting glucocorticoids only if severe
20%, persist to chronic form, these kids usually do not have viral prodrome

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25
Q

drug induced thrombocytopenia

A

quiniine
quinidine
vancomycin
all bind platelet glycoproteins and recognized as Ags
also seen in platelet inhibitory drugs that bind glycoprotein IIb/IIIa
HIT

26
Q

HIT

A

heparin induced thrombocytopenia

5% of people who receive heparin

27
Q

HIT type I

A

rapid onset after heparin administration
little clinical importance
dont discontinue heparin

28
Q

HIT type II

A

less common
5-14 days after therapy begins
life threatening thrombosis
Abs recognize heparin and platlet factor 4 complexes

29
Q

HIV-associated thrombocyotpenia

A

one of most common hematological manifestations of HIV
CD4 and CXCR4 (R and coR for HIV) found on megakaryocytes -> once infected prone to apoptosis
HIV also causes B-cell hyperplasia increasing probability of autoAbs

30
Q

thrombotic microangiopathies

A

caused by insults that lead to excessive platelet activation
intravascular thrombi cause microangiopathic hemolytic anemia
widespread organ dysfunction
thrombocytopenia due to consumption of platlets
unlike DIC activation of coagulation cascade, not primary importance therefore PT and PTT normal

31
Q

TTP

A

thrombotic thrombocytopenic purpura
pentad of fever, thrombocytopenia, micorangiopathic hemolytic anemia, transient neurological deficits, renal failure
not all 5 must be present
ADAMTS13 deficient (acquired more common then hereditary)
plasmaphoresis

32
Q

ADAMTS13

A

vWF metalloprotese

33
Q

HUS

A 
micorangiopathic hemolytic anemia
thrombocytopenia
acute renal failure
may have fever and neuro issues
children
normal ADAMTS13
34
Q

typical HUS

A

E. coli H7 -> shigga like toxin

children and older adults

35
Q

atypical HUS

A 
defects in C' factor H
cofactor protein CD46
factor I
all of these usually prevent excessive activation of alternative C' pathway
can be seen in pregnancy
36
Q

bleeding disorders related to platelet dysfunction categories

A

defects of adhesions
defects of aggregations
disorders in platelet secretion
acquired

37
Q

bernard-soulier syndrome

A

defect in adhesions of platelets to subendothelial matrix
inherited deficiency of Ib-IX
variable, often severe bleeding

38
Q

glanzmann thrombasthenia

A

autosomal recessive
deficiency of IIb-IIIa
bleeding often severe

39
Q

disorders of platelet secretion

A

storage pool disorders

40
Q

acquired bleeding disorders related to platelet dysfunction

A

ingestion of aspirin and other NSAIDs

Uremia

41
Q

hemorrhagic diatheses related to abnormalities in clotting factors

A

often occurs into GI and urinary tract and weight bearing joints
Vit K deficiency - II, VII, IX, X, protein C
DIC- all factors used up

42
Q

VIII

A

essential cofactor for activation of X
made in endothelium and kupffer cells
in circulation binds vWF which increases its half life
measured w/coagulation assays mixing patients plasma and factor VIII- deficient plasma

43
Q

vWF

A

produced by endothelium and megakarocytes
stabilizes VIII
measured w/ristocetin agglutination test where ristocetin cofactor would be decreased

44
Q

von Willebrand disease

A

most common inherited bleeding disorder of humans
bleeding tendancy is usually mild, often goes unnoticed until trauma
autosomal dominant
3 types

45
Q

vW disease types 1 and 3

A 
quantitative defects
autosomal domiinant
make up 70% of cases
type 1 mild bleeding
type 3 severe deficiency -> makes VIII unstable -> severe bleeding
prolonged PTT
46
Q

type 2

A

normal amount of vWF, dysfunctional
2a most common
autosomal dominant
mild-moderate bleeding

47
Q

hemophilia A

A

factor VIII disease
most common hereditary disease associated w/life threatening bleeding
x-linked recessive
30% acquired
severe disease
1-5% VIII moderate disease
>5% mild disease
easy bruising, massive hemorrhage from trauma
spontaneous bleeding into joints (hemarthroses)
petechiae usually absent
prolonged PTT and normal PT

48
Q

hemophilia B

A 
christmas disease
factor IX deficiency
clinically indistinguishable from hemophilia A
X-linked recesive 
PTT prolonged and normal PT
49
Q

DIC

A

acute, subacute, or chornic thrombohemorrhagic disorder characterized by excessive activation of coagulation and formation of thrombi in microvasculature
not a primary disease

50
Q

normal clotting

A

initiated by exposure of tissue factor which combines w/factor VII to activate factor X and IX -> thrombin -> converts fibrinogen -> fibrin -> feeds back and activated IX, VIII and V, stimulates cross linking of fibirn -> stabilizes clot

51
Q

thrombin

A

swept away from site of injury and converted to anticoagluant by binding thrombomodulin -> activates protein C -> inhibits V and VIII

52
Q

2 mechanisms for DIC

A

release of TF or other procoagulant

widespread injury to endothelium

53
Q

release of TF or other procoagulants

A

placenta in obstetric complications (most commoni)
trauma or burns
mucous from certain adenocarcinomas

54
Q

morphology of DIC

A

bilateral renal cortical necorsis if severe
fibrin degradation products
alveolar thrombi-> pulmonary edema -> hyaline membranes -> ARDS

55
Q

endocrine syndromes and DIC

A

waterhouse-friderichsen syndrome

kasaback-merrti syndrome

56
Q

clinical DIC

A

acute DIC - bleeding

chronic DIC thrombotic

57
Q

febrile nonhemolytic reaction

A

most common complication of transfusion
fevers, chills
sometimes mild dyspnea

58
Q

allergic rxns to transfusion

A

IgA deficient patients- severe, rare IgG binds donor IgA

urticarial rxns- allergen in donor blood recongnized by IgE, generally mild, do not need to discontinue transfusion

59
Q

acute hemolytic reactions

A

usually typing or labeling error -> mismatched blood type
fever, shaking, chills, flank pain
positive direct coombs test
can be fatal

60
Q

delayed hemolytic rnxs

A 
IgG
positive direct coombs
low haptoglobin and elevated LDH 
Ags: Rh, Kell, Kidd
can be as sever as blood type mismatch
61
Q

TRALI

A

transfusion related acute lung injury
severe frequently fatal rxn that activates neutrophils in lungs
rare, but more common in lung disease
2-hit hypothesis
Abs often from multiparous women
more likely with fresh frozen plasma and platelets
pulonary edema that does not respond to diuretics

62
Q

infectious complications

A

most bacterial arise from skin flora

significant bacterial contamination ore common in platelets

Heme II (7 decks)

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