Ch 14 - RBC & Bleeding Disorders Flashcards
1
Q
Principle clinical features of extravascular hemolysis
A
Jaundice, anemia, splenomegaly
2
Q
Cause by alternations that render RBCs less deformable
A
Extravascular hemolysis
3
Q
People with extravascular hemolysis often benefit from
A
Splenectomy
4
Q
Common causes of intravascular hemolysis (4)
A
Mechanical injury, complement fixation, intracellular parasites, exogenous toxic factors
5
Q
Intracellular hemolysis manifests as
A
Anemia, hemoglobinuria, hemoglobinemia, hemosiderinuria, jaundice
6
Q
Red brown urine from accumulation of Hb within renal tubular cells
A
Renal hemosiderosis
7
Q
Jaundice in uncomplicated hemolytic anemias is caused by
A
Unconjugated bilirubin
8
Q
Hereditary spherocytosis inheritance pattern
A
AD
9
Q
Small, dark-standing (hyperchromic) red cells lacking a central zone of pallor
A
Spherocytes
10
Q
Aplastic crisis ML caused by
A
Acute parvovirus infection
11
Q
Hemolytic crisis ML caused by
A
Infectious mono
12
Q
Blue-red polychromatophilic cytoplasm
A
Reticulocytes
13
Q
G6PD deficiency inheritance pattern
A
X-linked recessive
14
Q
MC trigger of G6PD def hemolytic episodes
A
Oxidative stress via infection: viral hepatitis, typhoid fever, pneumonia
15
Q
G6PD is characterized by
A
Episodic hemolysis when exposed to an oxidant
16
Q
Spleen infarction fibrosis and spleen progressively shrinks
A
Auto splenectomy - seen in HbSS
17
Q
MC sites of vaso-occlusive crises in HbSS pts
A
Born, liver, brain, spleen, penis
18
Q
Fever, cough, chest pain, pulmonary infiltration in HbSS pt
A
Acute chest syndrome
19
Q
Tx of HbSS
A
Hydroxyurea
20
Q
Beta-globin chain is on ch
A
11
21
Q
alpha-globin chain is on ch
A
16
22
Q
clinical manifestations of HbSS can be seen
A
5-6 mo after birth
23
Q
Clinical manifestations of beta-thal major can be seen
A
6-9 mo after birth
24
Q
RBCs contain little to no HbA and markedly elevated HbF
A
Beta-that major
25
Gamma chain tetramers,
Hb Barts
26
Beta chain tetramers
HbH
27
Fetal pallor, generalized edema, and massive HSM
Hydrops fetalis
28
Aquired mutations to PIGA
paroxysmal nocturnal hemoglobinuria
29
GIP protein that prevents the spontaneous activation of the alternative complement pathway by inhibiting C3 convertase
Membrane inhibitor or reactive lysis aka CD59
30
Leading cause of disease related death in PNH pts
Thrombosis
31
MC sites for thrombi in PNH pts
Hepatic, portal, and cerebral vv
32
Reduces the risk of thrombosis in PNH pts by 90%
Eculizumab
33
MOA: eculizumab
Inhibitor that prevents the conversion of C5 to C5a
34
MC type of autoimmune hemolytic anemia
Warm antibody type
35
MC causative Ab in warm antibody type IHA
IgG
36
MC antigenic drugs that cause warm ab type IHA
Penicillins and cephalosporins
37
IgM antibodies that bind red cells at v low temp
Cold agglutinin type IHA
38
MC infections that trigger cold agglutinin type IHA
Mycoplasma, EBV, CMV, influenza virus, HIV
39
Chronic cold agglutinin IHA ass'd with
B cell neoplasms or idiopathic
40
Cold hemolysis type IHA is mediated by
IgG to P blood group Ag on RBCs
41
IHA that can lead to potentially fatal intravascular hemolysis and hemoglobinuria
Cold hemolysin type
42
Microangiopathic hemolytic anemia is MC seen in
DIC, TTP, HUS, malignant HTN, SLE, and disseminated cancers
43
Salivary protein that binds B12 in the stomach
Haptocorrin
44
Receptor for IF in the ileum
Cubilin
45
Major carrier of B12 in the serum
Transcobalamin II
46
Blocks binding of B12 to IF
Type 1 Ab
47
Prevents binding of IF-B12 to ideal receptor
Type 2 Abs
48
Recognizes alpha and beta subunits of the gastric proton pump
Type 3 Abs
49
Pernicious anemia is often ass'd with
Autoimmune thyroiditis and adrenalitis
50
Atrophic glossitis, fundic gland atrophy with intesinalization, spastic paresis, sensory ataxia, LE parathesias
Pernicious anemia
51
Serum Abs for IF
Pernicious anemia
52
Gastric aphasia and metaplasia = increased risk for
Gastric carcinoma
53
Increase homocysteine levels = increased risk of
Atherosclerosis and thrombosis
54
Folate abs occurs in
Proximal jejunum
55
B12 is abs in
Ileum
56
Drugs that interfere with folic acid absorption
AEDs (phenytoin), OCs
57
Iron is absorbed in the
Duodenum
58
Iron absorption is regulated by
Hepcidin
59
Hepcidin is synthesized in the
Liver
60
- esophageal webs
- microcytic hypochromic anemia
- atrophic glossitis
Plummer Vinson Syndrome
61
Inflammatory mediator that stimulates hepatic production of Hepcidin
IL-6
62
MC known causes of aplastic anemias
Drugs, whole body irradiation, telomerase defects, Fanconi anemia
63
inheritance pattern of Fanconi's anemia
AR
64
Evidence of marrow hyperplasia early in life + multiple congenital anomalies
Fanconi anemia
65
Neoplasms ass'd with pure red cell aplasia
Thymoma, large granular lymphocytic lymphoma
66
Most cases of pure red cell aplasia have a basis of
Autoimmunity
67
Space occupying lesions replace normal marrow elements
Myelophthisic anemia
68
MCC of Myelophthisic anemia
Mets from breast, lung, or prostate
69
Endocrine disorder ass'd with mild n/n anemia
Hypoparathyroidism
70
common infectious cause of petechiae
Meningococcemia
71
Collagen defects that weaken BV walls
Survey, Ehlers Danlos
72
Colickey abd pain, purpuretic skin rash, polyarthralgia, acute GNP
Henoch-scholein purpura
73
AD disease with frequent epistaxis
Hereditary hemorrhagic telangiectasia
74
Thrombocytopenia is when platelet counts are less than
100k
75
Causes of thrombocytopenia (4)
Decreased platelet production, decreased platelet survival, platelet sequestration, blood dilution
76
MC causes of secondary chronic ITP
SLE, HIV, B cell neoplasms (lymphocytic leukemia)
77
ITP autoantibodies are directed at
Platelet membrane glycoproteins IIb-IIIa or Ib-IX
78
Large platelets in PBS, megakaryocytes increased in BM, small hemorrhages into mucous membranes throughout body, melena, hematuria, or excessive menstruation, platelets
chronic ITP
79
Thrombocytopenia that appears in kid 1-2 wks post viral illness and resolves w/in 6 mo
Acute ITP
80
MC drugs that cause drug-induced thrombocytopenia
Quinine, quinidine, vancomycin
81
Life threatning venous or arterial thrombosis occurring 5-14 d post heparin therapy
Type 2 HIT
82
Thrombocytopenia occurring rapidly after induction of heparin therapy
Type 1 - not as severe as type 2
83
Deficiency of ADAMTS13
TTP
84
Adolescent onset of TTP with episodic s/s
Hereditary TTP
85
Ass'd with complement factor H defects (CD 46)
Atypical HUS
86
Ass'd with shiga-like toxin, MC in kids
Typical HUS
87
Inherited disorder with defective platelet glycoprotein complex Ib-IX
Bernard-Soulier syndrome
88
Inherited disorder that leads to deficiency or dysfunction of platelet glycoproteins IIb-IIIa
Glanzmann thromboasthenia
89
Two clinically significant acquired defects in platelet function
NSAID (ASA) ass'd and uremia
90
Ristocetin agglutination test
Evaluates vWF function
91
Inheritance of Von Willebrand dz
AD
92
Type of Von willebrand dz with severe clinical manifestations
Type 3
93
Inheritance of type 3 Von willebrand dz
AR
94
Pretreat Von willebrand pts with
Desmopression
95
Inheritance of hemophilia A
X-linked recessive
96
Prolonged PTT and normal PT point to issues with the
Intrinsic pathway
97
Inheritance pattern of hemophilia B
X-linked recessive
98
How can you tell the diff btwn Hemophilia A & B?
Factor level assay
99
Two major mechanisms that trigger DIC
Release of TD or other procoaggulants into the circulation and widespread injury to endothelial cells
100
Up regulate expression of TF and adhesion molecules on endothelial cells
TNF
101
DIC is ML to be ass'd with (4)
Obstetric complications, malignant neoplasms, sepsis, major trauma
102
DIC ass'd with giant hemangiomas
Kasabach-Merritt syndrome
103
Cleaves very high molecular weight multimers of Von Willebrand factor
ADAMTS13
104
Systemic activation of coagulation
DIC
105
Chills, fever, mild dyspnea within 6 hr of transfusion
Febrile nonhemolytic reaction
106
Febrile nonhemolytic reactions are mediated by
Inflammatory mediators derived from donor leukocyte so
107
Allergic reactions to transfusions are ML in
Pts with IgA deficiency
108
Fever, chills, shaking, flank pain, + direct Coombs after blood transfusion
Hemolytic reaction - IgM mediated
109
Sudden, dramatic onset of respiratory failure following transfusion
TRALI
110
Ab MCly ass'd with TRALI
MHC Class 1 - MC in multiparous females
111
Coombs - hemolytic anemia, pancytopenia, venous thrombosis
Paroxysmal nocturnal hemoglobinuria
112
5-10% of PNH pts develop
AML or MDS
113
Decreased serum iron, increased TBIC, decreased ferritin
Fe def anemia
114
Decreased serum iron, decreased TBIC, increased ferritin
Anemia of chronic disease
115
increased serum iron, decreased TBIC, increased ferritin
Hemochromatosis
116
Increased TBIC, decreased transferrin saturation
Pregnancy/ OC use
117
TMPRSS6 mutation
no hepcidin suppression
