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Heme & Lymph > Ch 14 - RBC & Bleeding Disorders > Flashcards

Ch 14 - RBC & Bleeding Disorders Flashcards

1
Q

Principle clinical features of extravascular hemolysis

A

Jaundice, anemia, splenomegaly

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2
Q

Cause by alternations that render RBCs less deformable

A

Extravascular hemolysis

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3
Q

People with extravascular hemolysis often benefit from

A

Splenectomy

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4
Q

Common causes of intravascular hemolysis (4)

A

Mechanical injury, complement fixation, intracellular parasites, exogenous toxic factors

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5
Q

Intracellular hemolysis manifests as

A

Anemia, hemoglobinuria, hemoglobinemia, hemosiderinuria, jaundice

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6
Q

Red brown urine from accumulation of Hb within renal tubular cells

A

Renal hemosiderosis

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7
Q

Jaundice in uncomplicated hemolytic anemias is caused by

A

Unconjugated bilirubin

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8
Q

Hereditary spherocytosis inheritance pattern

A

AD

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9
Q

Small, dark-standing (hyperchromic) red cells lacking a central zone of pallor

A

Spherocytes

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10
Q

Aplastic crisis ML caused by

A

Acute parvovirus infection

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11
Q

Hemolytic crisis ML caused by

A

Infectious mono

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12
Q

Blue-red polychromatophilic cytoplasm

A

Reticulocytes

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13
Q

G6PD deficiency inheritance pattern

A

X-linked recessive

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14
Q

MC trigger of G6PD def hemolytic episodes

A

Oxidative stress via infection: viral hepatitis, typhoid fever, pneumonia

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15
Q

G6PD is characterized by

A

Episodic hemolysis when exposed to an oxidant

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16
Q

Spleen infarction fibrosis and spleen progressively shrinks

A

Auto splenectomy - seen in HbSS

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17
Q

MC sites of vaso-occlusive crises in HbSS pts

A

Born, liver, brain, spleen, penis

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18
Q

Fever, cough, chest pain, pulmonary infiltration in HbSS pt

A

Acute chest syndrome

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19
Q

Tx of HbSS

A

Hydroxyurea

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20
Q

Beta-globin chain is on ch

A

11

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21
Q

alpha-globin chain is on ch

A

16

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22
Q

clinical manifestations of HbSS can be seen

A

5-6 mo after birth

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23
Q

Clinical manifestations of beta-thal major can be seen

A

6-9 mo after birth

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24
Q

RBCs contain little to no HbA and markedly elevated HbF

A

Beta-that major

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25
Q

Gamma chain tetramers,

A

Hb Barts

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26
Q

Beta chain tetramers

A

HbH

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27
Q

Fetal pallor, generalized edema, and massive HSM

A

Hydrops fetalis

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28
Q

Aquired mutations to PIGA

A

paroxysmal nocturnal hemoglobinuria

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29
Q

GIP protein that prevents the spontaneous activation of the alternative complement pathway by inhibiting C3 convertase

A

Membrane inhibitor or reactive lysis aka CD59

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30
Q

Leading cause of disease related death in PNH pts

A

Thrombosis

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31
Q

MC sites for thrombi in PNH pts

A

Hepatic, portal, and cerebral vv

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32
Q

Reduces the risk of thrombosis in PNH pts by 90%

A

Eculizumab

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33
Q

MOA: eculizumab

A

Inhibitor that prevents the conversion of C5 to C5a

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34
Q

MC type of autoimmune hemolytic anemia

A

Warm antibody type

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35
Q

MC causative Ab in warm antibody type IHA

A

IgG

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36
Q

MC antigenic drugs that cause warm ab type IHA

A

Penicillins and cephalosporins

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37
Q

IgM antibodies that bind red cells at v low temp

A

Cold agglutinin type IHA

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38
Q

MC infections that trigger cold agglutinin type IHA

A

Mycoplasma, EBV, CMV, influenza virus, HIV

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39
Q

Chronic cold agglutinin IHA ass’d with

A

B cell neoplasms or idiopathic

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40
Q

Cold hemolysis type IHA is mediated by

A

IgG to P blood group Ag on RBCs

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41
Q

IHA that can lead to potentially fatal intravascular hemolysis and hemoglobinuria

A

Cold hemolysin type

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42
Q

Microangiopathic hemolytic anemia is MC seen in

A

DIC, TTP, HUS, malignant HTN, SLE, and disseminated cancers

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43
Q

Salivary protein that binds B12 in the stomach

A

Haptocorrin

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44
Q

Receptor for IF in the ileum

A

Cubilin

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45
Q

Major carrier of B12 in the serum

A

Transcobalamin II

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46
Q

Blocks binding of B12 to IF

A

Type 1 Ab

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47
Q

Prevents binding of IF-B12 to ideal receptor

A

Type 2 Abs

48
Q

Recognizes alpha and beta subunits of the gastric proton pump

A

Type 3 Abs

49
Q

Pernicious anemia is often ass’d with

A

Autoimmune thyroiditis and adrenalitis

50
Q

Atrophic glossitis, fundic gland atrophy with intesinalization, spastic paresis, sensory ataxia, LE parathesias

A

Pernicious anemia

51
Q

Serum Abs for IF

A

Pernicious anemia

52
Q

Gastric aphasia and metaplasia = increased risk for

A

Gastric carcinoma

53
Q

Increase homocysteine levels = increased risk of

A

Atherosclerosis and thrombosis

54
Q

Folate abs occurs in

A

Proximal jejunum

55
Q

B12 is abs in

A

Ileum

56
Q

Drugs that interfere with folic acid absorption

A

AEDs (phenytoin), OCs

57
Q

Iron is absorbed in the

A

Duodenum

58
Q

Iron absorption is regulated by

A

Hepcidin

59
Q

Hepcidin is synthesized in the

A

Liver

60
Q
  • esophageal webs
  • microcytic hypochromic anemia
  • atrophic glossitis
A

Plummer Vinson Syndrome

61
Q

Inflammatory mediator that stimulates hepatic production of Hepcidin

A

IL-6

62
Q

MC known causes of aplastic anemias

A

Drugs, whole body irradiation, telomerase defects, Fanconi anemia

63
Q

inheritance pattern of Fanconi’s anemia

A

AR

64
Q

Evidence of marrow hyperplasia early in life + multiple congenital anomalies

A

Fanconi anemia

65
Q

Neoplasms ass’d with pure red cell aplasia

A

Thymoma, large granular lymphocytic lymphoma

66
Q

Most cases of pure red cell aplasia have a basis of

A

Autoimmunity

67
Q

Space occupying lesions replace normal marrow elements

A

Myelophthisic anemia

68
Q

MCC of Myelophthisic anemia

A

Mets from breast, lung, or prostate

69
Q

Endocrine disorder ass’d with mild n/n anemia

A

Hypoparathyroidism

70
Q

common infectious cause of petechiae

A

Meningococcemia

71
Q

Collagen defects that weaken BV walls

A

Survey, Ehlers Danlos

72
Q

Colickey abd pain, purpuretic skin rash, polyarthralgia, acute GNP

A

Henoch-scholein purpura

73
Q

AD disease with frequent epistaxis

A

Hereditary hemorrhagic telangiectasia

74
Q

Thrombocytopenia is when platelet counts are less than

A

100k

75
Q

Causes of thrombocytopenia (4)

A

Decreased platelet production, decreased platelet survival, platelet sequestration, blood dilution

76
Q

MC causes of secondary chronic ITP

A

SLE, HIV, B cell neoplasms (lymphocytic leukemia)

77
Q

ITP autoantibodies are directed at

A

Platelet membrane glycoproteins IIb-IIIa or Ib-IX

78
Q

Large platelets in PBS, megakaryocytes increased in BM, small hemorrhages into mucous membranes throughout body, melena, hematuria, or excessive menstruation, platelets

A

chronic ITP

79
Q

Thrombocytopenia that appears in kid 1-2 wks post viral illness and resolves w/in 6 mo

A

Acute ITP

80
Q

MC drugs that cause drug-induced thrombocytopenia

A

Quinine, quinidine, vancomycin

81
Q

Life threatning venous or arterial thrombosis occurring 5-14 d post heparin therapy

A

Type 2 HIT

82
Q

Thrombocytopenia occurring rapidly after induction of heparin therapy

A

Type 1 - not as severe as type 2

83
Q

Deficiency of ADAMTS13

A

TTP

84
Q

Adolescent onset of TTP with episodic s/s

A

Hereditary TTP

85
Q

Ass’d with complement factor H defects (CD 46)

A

Atypical HUS

86
Q

Ass’d with shiga-like toxin, MC in kids

A

Typical HUS

87
Q

Inherited disorder with defective platelet glycoprotein complex Ib-IX

A

Bernard-Soulier syndrome

88
Q

Inherited disorder that leads to deficiency or dysfunction of platelet glycoproteins IIb-IIIa

A

Glanzmann thromboasthenia

89
Q

Two clinically significant acquired defects in platelet function

A

NSAID (ASA) ass’d and uremia

90
Q

Ristocetin agglutination test

A

Evaluates vWF function

91
Q

Inheritance of Von Willebrand dz

A

AD

92
Q

Type of Von willebrand dz with severe clinical manifestations

A

Type 3

93
Q

Inheritance of type 3 Von willebrand dz

A

AR

94
Q

Pretreat Von willebrand pts with

A

Desmopression

95
Q

Inheritance of hemophilia A

A

X-linked recessive

96
Q

Prolonged PTT and normal PT point to issues with the

A

Intrinsic pathway

97
Q

Inheritance pattern of hemophilia B

A

X-linked recessive

98
Q

How can you tell the diff btwn Hemophilia A & B?

A

Factor level assay

99
Q

Two major mechanisms that trigger DIC

A

Release of TD or other procoaggulants into the circulation and widespread injury to endothelial cells

100
Q

Up regulate expression of TF and adhesion molecules on endothelial cells

A

TNF

101
Q

DIC is ML to be ass’d with (4)

A

Obstetric complications, malignant neoplasms, sepsis, major trauma

102
Q

DIC ass’d with giant hemangiomas

A

Kasabach-Merritt syndrome

103
Q

Cleaves very high molecular weight multimers of Von Willebrand factor

A

ADAMTS13

104
Q

Systemic activation of coagulation

A

DIC

105
Q

Chills, fever, mild dyspnea within 6 hr of transfusion

A

Febrile nonhemolytic reaction

106
Q

Febrile nonhemolytic reactions are mediated by

A

Inflammatory mediators derived from donor leukocyte so

107
Q

Allergic reactions to transfusions are ML in

A

Pts with IgA deficiency

108
Q

Fever, chills, shaking, flank pain, + direct Coombs after blood transfusion

A

Hemolytic reaction - IgM mediated

109
Q

Sudden, dramatic onset of respiratory failure following transfusion

A

TRALI

110
Q

Ab MCly ass’d with TRALI

A

MHC Class 1 - MC in multiparous females

111
Q

Coombs - hemolytic anemia, pancytopenia, venous thrombosis

A

Paroxysmal nocturnal hemoglobinuria

112
Q

5-10% of PNH pts develop

A

AML or MDS

113
Q

Decreased serum iron, increased TBIC, decreased ferritin

A

Fe def anemia

114
Q

Decreased serum iron, decreased TBIC, increased ferritin

A

Anemia of chronic disease

115
Q

increased serum iron, decreased TBIC, increased ferritin

A

Hemochromatosis

116
Q

Increased TBIC, decreased transferrin saturation

A

Pregnancy/ OC use

117
Q

TMPRSS6 mutation

A

no hepcidin suppression

Heme & Lymph (11 decks)

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