Week 1 RR Flashcards
1
Q
hypoxic injury and infarction w/ severe pain in the affected organ
A
Pain crises (vaso-occlusive)–> sickle cell disease
2
Q
Transfusion rxn: Fever, Chills, back pain and headache; hemoglobinuria, hemoglobinemia
A
Acute hemolytic transfusion reaction
3
Q
G6PD recovery phase
A
reticulocytosis.
4
Q
Sickle Cell Anemia inheritance
A
Autosomal recessive
5
Q
PIG-A is x-linked; significance?
A
when PIG-A is INACTIVATED–> PNH PIG-A may be inactivated through lyonization causing PNH
6
Q
hydrops fetalis pathogenesis
A
high output cardiac failure–> Liver dysfunction–> Dec albumin–> EDEMA
7
Q
pre-renal azotemia: oliguria or anuria
A
Acute blood loss
8
Q
s/s of HTLV‐1 and HTLV‐2
A
T‐Cell lymphoma Demyelinating neuropathy
9
Q
“bite cells
A
G6PD
10
Q
OLDER RBCs have more damage
A
G6PD
11
Q
what are Heinz bodies and where found
A
seen in G6PD def. Sulfhydryl groups in Hb is oxidized and precipitates forming Heinz bodies
12
Q
Seen in Middle Eastern ppl
A
Mediterranean variant G6PD
13
Q
secondary hemosiderosis (iron overload)
A
Thalassemia (from the transfusion) Sickle Cell anemia Hereditary spher.
14
Q
Megaloblastic Anemia retic count; MCV
A
macrocytic MCV>100 ↓ retic count
15
Q
PNH mutation
A
INACTIVATING somatic mutation of PIG-A
16
Q
acute vs. delayed Hemolytic transfus. rxn: intra/extravascular hemolysis
A
acute–> intra delayed–> extravascular
17
Q
Pernicious anemia diagnosis
A
anti-IF antibody
18
Q
NO levels in Sickle cell disease; significance?
A
it is depleted b/c Hb from lysed sickle cells bind it; Result–> more vasconstriction leading to INC transit time and more problems
19
Q
Erythroblastosis fetalis retic count
A
INC. because it is hemolytic
20
Q
the direct Coombs detects (1) and the indirect detects (2)
A
- antibodies on red cells 2. antibodies in serum or plasma
21
Q
Drug induced: Sudden, severe hemolysis w/ Hburia
A
Cephalosporins.
22
Q
associated w/ chronic inflammation
A
Anemia of Chronic Disease (ACD)
23
Q
intravascular hemolysis
A
G6PD PNH Microangiopathic Hemolytic Anemia
24
Q
IgG vs IgM in erythro. fetalis
A
IgG can cross placenta; IgM too large;
25
(a) ineffective erythropoiesis (b) extravascular hemolysis
β-thalassemia
26
RBCs susceptible to destruction by complement. \*i.e. decreased inhibition of complement activation\*
PNH
27
Acute blood loss lab values
↑ platelets (thrombocytosis) ↑ WBC esp. granulocyte (leukocytosis)
28
Pseudo‐Pelger –Huet cells--\>
dumb-bell shaped neutrophils seen in Myelodysplastic syndrome
29
↑serum homocysteine; ↑ methylmalonic acid
Vit. B12 def.
30
IgM antibodies bind to RBCs at less than 37 deg. C
Cold AIHA
31
how is metabolic complications in blood transfusion occur
Blood unit is anticoagulated w/ citrate; Rapid infusion chelates calcium
32
Manifestations of post transfusion purpura
severe bleeding thrombocytopenia 1-3 weeks post transfusion
33
HbC (Lys for Glu) cells lose 1) and become dehydrated, sickling
1) salt and water
34
hyper-segmented (\>5 nuclei) neutrophils
Megaloblastic Anemia
35
extravascular hemolysis
Hereditary spherocytosis Immune Hemolytic Anemia Thalassemia
36
1) inhibits HbS polymerization
1) HbF
37
P. pneumoniae and H. influenzae
Encapsulated organisms; seen in Sickle patients
38
Hypoalbuminemia Generalized edema
Fetal compensation in erythro. fetalis
39
(Paroxysmal Nocturnal Hburia) inheritance
ACQUIRED; not inherited
40
Febrile non hemolytic transfusion reactions are caused by (1) antibodies in the recipients plasma which react with (2) leading to the release of endogenous pyrogens (3)
1. anti‐leukocyte or antiplatelet 2. donor WBC’s 3. IL‐1 ,IL‐6 , TNF‐alpha
41
non-transmembrane proteins that attach to the cell membrane through GPI
PIGA
42
peripheral nucleated RBC’s, polychromatophylia (indicates ↑turnover), thrombocytopenia and ↑bilirubin
Erythroblastosis fetalis
43
Sickle cell pathogenesis: Deoxygenated HbS polymerizes--\> aggregate into long needle-like structures--\> ↓pH--\> 1)
1) RBC sickles
44
Hereditary Spherocytosis--\> intravascular or extravascular
Extravascular
45
(CD55
Decay-accelerating factor--\>inhibitor of complement
46
coats the Rh positive cells of fetus and prevents sensitization of mother
Rho-gam; only effective against Rh(D)
47
Direct Coomb’s test: (-) Anti-IgG (+) anti-C3d
Cold AIHA
48
decreased reticulocyte count
Iron def. anemia Thalassemia Megaloblastic anemia ACD Aplastic anemia
49
Sickle cell hallmark in children
Hand-foot syndrome--\> swollen hands and feet
50
Myelodysplastic syndrome: (1) gene is located on chromosome 8 and trisomy 8 is commonly seen in a variety of (2)
1. MYC 2. myeloid malignancies
51
Acute hemolytic transfusion reactions are most often from transfusion of (1
1. ABO incompatible red blood cells
52
β0-thalassemia vs. β+-thalassemia
β0-thalassemia: Absent functional β-globin protein β+-thalassemia: ↓ β-globin protein.
53
Sickle cell MCHC
Increased due to intracellular dehydration
54
Transfusion complication: HTN; SOB, pul. edema, etec.
Circulatory overload
55
Antigen antibody reactions from infusion of plasma proteins
Allergic Rxns to blood transfusion
56
IgM vs. IgG
Erythroblastosis fetalis: IgM can't cross placenta IgG can. Autoimmune Hemolytic anemia: warm--\> IgG Cold--\> IgM Drug Induced Hemolytic anemia:
57
Disposal of the oxidant H2O2 is dependent on reduced glutathione (GSH), which is generated by the action of 1); without G6PD, 1) cannot be generated
NADPH
58
SEVERE anaphylaxis due to transfusion
IgA deficient pts
59
This cytokine released during (1) binds to hemoglobin leading to renal vasoconstriction
1. acute hemolytic transfusion reactions; cytokine is NO (EDFR)
60
Myelodysplastic syndrome: 5q vs. 7q
5q--\> often good prognosis in older women 7q--\>worse prognosis
61
Point mutation in beta globiin gene;
β-thalassemia
62
Sicke cell diagnosis
Positive sickling w/ metabisulfate; Hb electrophoresis shows High HbS
63
More severe. Markedly reduced ½ life (reduction in protein stability
Mediterranean variant G6PD
64
anti-leukocyte or anti-platelet Abs in host plasma which react with donor WBCs
Febrile non hemolytic transfusion reaction
65
Cold agglutinins are associated with
mycoplasm pneumonia, infectious mono (EBV, CMV)
66
Transfusion complication: pt on ACE inhibitor (unable to break down bradykinin)
Hypotensive rxn;
67
Erythroid hyperplasia in the bone marrow (“crewcut” on scalp), extramedullary hematopoiesis (in liver, spleen, and lymph nodes).
Sickle cell
68
Drug induced: Direct or indirect Coombs' test: Complement only
immune-complex type
69
PNH--\> intravascular or extravascular
intravascular hemolysis
70
acute phase reactants such as ferritin and hepcidin ↑
Anemia of chronic disease (ACD)
71
INHERITED defect in RBC membrane
Hereditary Spherocytosis
72
Myelodysplastic syndrome has a high risk of transformation to (1)
Acute Myeloid Leukemia (AML).
73
Aplastic crises: RBC progenitors w/ parvovirus B19
Sickle cell disease
74
Antibodies against transfused leukocytes or platelets release endogenous pyrogens(IL‐1 ,IL‐6 , TNF‐alpha )
Febrile non hemolytic transfusion reaction:
75
hypocellular marrow with lots of fat; few erythroid progenitors
Aplastic Anemia
76
1 mutated β gene vs. 2 mutated
Thalssemia minor--\> Mild anemia 2: Thalassemia major--\> severe anemia; needs transfusion
77
PNH RBCs go through intravascular hemolysis caused by 1)
1) C5b-C9 MAC
78
small molecule that generates an immune response when attached to a larger molecule.
Hapten
79
↓haptoglobin.
Intravascular hemolysis
80
G6PD deficiency--\> intravascular or extravascu
intravascular hemolysis
81
Splenomegaly
Extravascular
82
colon polyps/carcinoma in elderly
Iron-Deficiency Anemia
83
Sickle cell anemia s/s due to extravascular hemolysis
Bilirubin stones;
84
seen in conditions such as Diabetes, RA, SLE, neoplastic disorders
Anemia of Chronic Disease (ACD)
85
Poikilocytosis vs. aniscocytosis
aniScocytosis--\> varying Sizes poikilocytosis--\> varying SHAPES
86
Iron-Deficiency Anemia lab
↓serum iron ↓Ferritin; ↑TIBC;
87
Erythroblastosis fetalis LABS
Inc. UCB; Dec. platelets (thrombocytopenia)
88
G6PD s/s
Intravascular hemolysis therefore: Hbemia; Hburia ANEMIA
89
Myelodysplastic syndrome: Epigenetic factors
hyper methylation with silencing of tumor suppressor genes
90
Sickle cell lab test
Mix blood with metabisulfate--\>
91
Mildly reduced ½ life of G6PD (protein misfolding)
African variant G6PD
92
most common cause of death in adults with sickle cell disease
Acute Chest Syndrome
93
Erythroblastosis fetalis: jaundice soon after birth. why not in utero
placenta handles the bilirubin in utero
94
extravascular hemolysis s/s
Splenomegaly, jaundice ↑risk of pigmented gallstones.
95
Hereditary Spherocytosis inheritance
AD; more severe when compound heterozygote
96
associated w/ PNH
Aplastic Anemia
97
Erythroblastosis fetalis direct coomb's test
(+) direct Coombs test (IgG)
98
Acquired antibody mediated RBC destruction
autoimm. hemolytic anemia
99
MCV, Hb, Hct and MCH are all ↓.
Iron-Deficiency Anemia
100
Drug induced: penicillin
Hapten Mechanism
101
Cause of death in PNH
venous Thrombosis--\> platelet pieces destroyed by compliment clump together;
102
What are the s/s of chelation of calcium?
Circumoral paresthesia and tingling of fingers
103
dumb-bell shaped neutrophils with two nuclei
Pseudo‐Pelger –Huet celll--\>Myelodysplastic syndrome
104
Intravascular and extravascular hemolysis
Sickle Cell disease
105
associate w/ ankyrin, band 4.2 and band 3.1; alpha and beta spectrin dimers
Hereditary Spherocytosis
106
Which transfusion reactions are complement mediated?
Acute hemolytic transfusion reactions Delayed are not complement mediated
107
an excess of α-globin chains causes problems
β-thalassemia (↓β-globin mRNA)
108
Secondary to mycoplasma pneumoniae infection
Cold AIHA
109
target cells, microcytic hypochromic cells
Sickle cell
110
↑serum homocysteine; normal methylmalonic acid
Folate def.
111
blood from multiparous woman can cause:
TRALI (Transfusion acute LUNG injurY)
112
loss of central pallor
Hereditary Spherocytosis
113
Inc. susceptibility to oxidative damage
G6PD
114
myeloblasts in myelodysplastic syndrome vs. acute myeloid leukemia
Myelodys--\> inc. but less than 20% AML--\> myeloblasts less than 20%
115
Transfusion complication: Hypotensive rxn; s/s
HYPOTENSION facial flushing and abdominal pain
116
Acute Chest Syndrome s/s
chest pain, and pulmonary infiltrates; SOB; seen in Sickle cell pts
117
fava beans
G6PD--\> generates oxidants when metabolized
118
hemoglobinemia, hemoglobinuria, hemosiderinuria
intravascular hemolysis
119
Transfusion complication: HBV vs. HCV
HBV--\> jaundice 2 mos after; very few become chronic carrier HCV--\> non-icteric; HIGH percent becomes chronic carriers
120
Transfusion complication in an IgA def. pt
anaphylaxis hypotension
121
Thalassemia--\> intravascular or extravascular
Extravascular
122
Dec. pH on Sickle cell
INCREASES sickling
123
β-Thalassemia major vs. minor genetics
Major--\>Homozygous (some compound heterozygous) minor--\> heterozygous
124
vitamin B12 or Folic acid deficiencies
Megaloblastic Anemia
125
Sickle cell on xray; why?
crewcut appearance due to compensatory erythroid hyperplasia in the bone marrow of SKULL
126
Why should neonates get blood 7 day old or less for exchange transfusions?
To reduce the risk of a storage lesion in which potassium leaks out of RBC during storage
127
Drug induced: Drug stimulates IgM production against RBCs
Immune Complex Mechanism
128
If store blood for too long, what may occur?
Metabolic Complications: Storage lesion --\> K+ leaks out of RBC during storage
129
“Underhemoglobinized” hypochromic microcytic cells
β-thalassemia
130
Transfusion related acute lung injury is due to antibodies in the donor plasma directed against the recipient’s (1); these are usually (2) antigens
1. WBC’s 2. class I MHC
131
Hburia in the morning that disappears in the afternoon
Paroxysmal Nocturnal Hburia
132
Sulfhydryl groups in Hb is oxidized and precipitates forming 1)
1) Heinz bodies
133
G6PD inheritance
X-linked recessive
134
metabisulfate test
causes sickling in both sickle cell disease and trait
135
Due to slow transit times, Sickled cells show increased adhesion 1)
1) to vascular endothelium
136
Increased risk of sickling occurs w/
hypoxemia, dehydration and acidosis
137
Drug induced: Direct or indirect Coombs' test: IgG only
hapten type or autoantibody type
138
What problem can occur as a result of a rapid infusion?
Rapid infusion results in chelation of calcium by citrate (which is used as an anticoagulant for blood)
139
↑ LDH
seen in hemolysis
140
Direct Coomb’s Test: (+) anti-IgG, (+) anti-C3d or both.
Warm AIHA
141
Sickle cell: polymers aggregate and RBCs lose 1) and have 2)pH;
1) intracellular K+ and h2O 2) ↓
142
Erythroblastosis fetalis s/s
ANEMIA hepato-splenomegaly; jaundice soon after birth; Generalized edema;
143
common infection in Sickle cell
Osteomyelitis w/ salmonella or other encapsulated organisms
144
CD59
inhibits C3 convertase and activation of the alternative complement pathway.
145
HPA-1a; what is it?
if pt lacks this, after transfusion, develops post transfusion purpura;
146
Fever; urticaria, pruritus, erythema after transfuion
allergic reaction
147
↑RDW
Iron def. anemia Hereditary spherocytosis
148
cell is expanding in size but nucleus is not dividing—hence macrocytic
Megaloblastic Anemia
149
Transfusion complication: pt LACK platelet antigen HPA-1a
post transfusion purpura
150
affect of pH on sickling
dec. pH reduces the oxygen affinity of hemoglobin, thereby increasing the fraction of deoxygenated HbS at any oxygen level increasing sickling
151
Pernicious anemia lab
↓ Intrisic factor ↓ Vitamin B12 therefore ↑serum homocysteine; ↑ methylmalonic acid
152
↑ risk of parvovirus--\> aplastic crisis
Thalassemia Hereditary Spher. Sickle Cell
153
IgG abs opsonize RBCs at body temperature
warm AIHA
154
Pt with CHF or heart failure--\> what transfusion complication?
Circulatory overload
155
Splicing mutations --\> create an “ectopic” cryptic splice site within the intron
β+-thalassemia
156
Sickle Cell Anemia: amino acid change
Glutamic acid replaced by Valine
157
Destruction of splice donor or splice acceptor sites.
β0-thalassemia
158
hyper cellular bone marrow with ringed sideroblasts,
Myelodysplastic syndrome:
159
Structurally abnormal hemoglobin; quantity fine
Sickle Cell Anemia
160
Increased LDH and UCB, decreased Hct and haptoglobin; Hemoglobin normal!
Delayed hemolytic transfusion rxn
161
Promotor mutations--\> ↓ transcription (i.e. affects pre-mRNA)
β+-thalassemia
162
conditions that decrease the MCHC (alpha thalassemia) reduce the disease severity
Sickle cell
163
Hand-foot syndrome: Dactylitis of bones—happens in children
Sickle cell
164
Hereditary Spherocytosis s/s
extravascular hemolysis therefore: ↑ UCB--\> jaundice Bilirubin gallstone ANEMIA ↑LDH
165
att birth w/ marked jaundice and require transfusions.
compound heterozygote Hereditary Spherocytosis
166
Hereditary Spherocytosis treatment:
Splenectomy
167
compound heterozygote Hereditary Spherocytosis
birth w/ marked jaundice and require transfusions.
168
protected against P. falciparum malaria
Sickle Cell anemia Thalassemia G6PD
169
Febrile non hemolytic transfusion reaction labs
normal; no inc. LDH, UCB, Retic, count, no decrease in haptoglobin or Hct
170
monnuclear megakaryocytes with no platelets in periphery
Myelodysplastic syndrome:--\> dysmegakaryopoiesis
171
How does transfusion related acute lung injury appear on chest x-ray?
diffuse bilateral pulmonary infiltrates which are unresponsive to diuretics
172
Oxidant drugs --\> antimalarials, sulfonamids, and nitrofurantoins.
G6PD
173
Target cells
Thalassemia Sickle cell
174
decreased synthesis of the globin chains of Hb
Thalassemia
175
Thalassemia retic count
↑ Reticulocyte count (extravascular hemo)
176
Nonsense or frameshift mutations--\> early stop codon
β0-thalassemia
177
Homozygous for HbS but also has coexistent 1), which reduces Hb synthesis and has milder disease
1) α-thalassemia
178
Drug induced: Drug stimulates IgG production against RBCs
Autoantibody Mechanism
179
Hereditary Spherocytosis diagnosis:
Osmotic fragility test: red cells lyse in hypotonic sln
180
microcytic, hypochromic RBCs, target cells, and basophilic stippling
β-thalassemia
181
Complement mediated RBC lysis, platelet aggregation, and hypercoagulability
PNH
182
Iron-Deficiency Anemia RBC indices
MCV, Hb, Hct and MCH are all ↓.
183
Vascular obstruction, particularly stroke (CVA)
Sickle cell disease
184
INACTIVATING somatic mutation of PIG-A
PNH
185
increased reticulocyte count
Immune Hemolytic Anemia Hereditary Spherocytosis G6PD deficiency PNH
186
Sickle cells s/s due to vasoocclusion
vascular necrosis of the femur renal infarcts Not comprehensive
187
PNH diagnosis
Deficient: Decay-accelerating factor (CD55 Membrane inhibitor of lysis (CD59) C8 binding protein
188
acquired clonal stem cell disorder; DYSMORPHIC blood cells and precursors
Myelodysplastic syndrome:
189
Sickling of RBCs either cause:
(a) Hemolysis: extravascular (spleen) (b) Microvascular occlusion (due to sickle shape)
190
Transfusion complication: Acute hemolytic transfusion rxn: what cytokines?
Pro inflammatory cytokines (IL‐1 ,IL‐6 ,IL‐8 ) TNF alpha NO (EDFR )
191
Sickle Cell Trait vs. disease inheritance
Sickle Cell Trait --\> heterozygous Sickle Cell Disease --\> homozygous
192
asymptomatic—only sickle on profound hypoxia
Sickle Cell Trait
193
Autoimmune Hemolytic Anemia intra/extravascular
EXTRAvascular; both warm and cold
194
Intravascular hemolysis s/s
Hbemia, Hburia, Hemosiderinuria, and ↓haptoglobin
195
Delayed hemolytic transfusion rxn lab
lack of expected rise in Hb;
196
ACD RBC indices
Normocytic normochromic
197
Drug induced: Mild to moderate hemolysis, w/ slow onset developing over days to weeks
• Penicillin • Methyldopa
198
Erythroblastosis fetalis complication
kernicterus due to Inc. UCB (fat soluble)
199
β-thalassemia Hb electrophoresis
↑in HbA2 (α2δ2)
200
Sickle cell: 1) in the sixth codon of β-globin that leads to the replacement of a glutamate with a valine residue.
1) point mutation
201
β-Thalassemia: ___ is temporarily protective
HbF
