normocytic anemias Flashcards

1
Q

normocytic anemia

A
  • 80-100 MCV
  • issue with destruction, production, kidneys, or malignancy
  • peripheral smear is key
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

causes of normocytic anemias

A
  • heomlytic anemia
  • hereditary spherocytosis
  • autoimmune hemolytic anemia
  • cold agglutin disease
  • paroxysmal nocturnal hemaglobinuria
  • G6PD def
  • sickle cell disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

why does anemia occur with continued hemolysis

A
  • extremely short RBC survival

- ability of bone marrow to compensate is impaired

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

intrinsic/ inherited causes of hemolytic anemia

A
  • genes that control how we make RBCs are abnormal
  • membrane defects
  • enzyme deficiency/ oxidation vulnerability
  • hemoglobinopathies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

acquired/ extrinsic causes of hemolytic anemia

A
  • something else destroying the cells
  • immune: autoimmune, drug toxicity
  • microangiopathic
  • infection
  • hypersplenism
  • burn
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

si/sx of hemolytic anemia

A
  • fatigue, weakness, pallor
  • easy bruising, petechiae, purpura
  • yellow discoloration of eyes and skin
  • abd pain
  • dark urine
  • splenomegaly
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

lab findings for hemolytic anemia

A
  • bilirubin accumuation d/t heme breakdown
  • increased retic count
  • decreased haptoglobin
  • pos urine hemosiderin
  • increased serum LDH
  • increased methemalbuminemia and hemoglobinemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

hereditary spherocytosis

A
  • abnormal spectrin and ankyrin (abnormal cell membrane)
  • causes small, hyperchromic, inflexible cells
  • get trapped in spleen and hemolyzed
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

key findings for hereditary spherocytosis

A
  • family hx
  • splenomegaly
  • spherocytosis and reticulocytes on smear
  • may or may not be anemic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

diagnosis of hereditary spherocytosis

A
  • spherocytosis
  • familial hemolytic anemia
  • abnormal osmotic fragility
  • coombs negative
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

treatment of hereditary spherocytosis

A
  • supportive care
  • transfusions as needed
  • folic acid cupplementation
  • consider splenectomy if sx are severe
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

paroxysmal nocturnal hemoglobinuria (PNH)

A
  • rare hematopoietic stem cell disorder
  • abnormal sensitivity of RBC membranes to lysis by complement
  • lack of CD55 and CD59 proteins
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is the most common cause of death in PNH

A
  • thrombosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

si/sx of PNH

A
  • episodic hemoglobinuria- reddish brown urine usu first thing in AM
  • fatigue, weakness
  • esophageal spasms
  • ED
  • abd pain
  • dyspnea
  • thrombosis**
  • pulm HTN
  • end organ damage, CKD
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

common sites of thrombosis in PNH

A
  • mesenteric v
  • hepatic v
  • CNS veins
  • skin vessels- formation of painful nodules
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

lab findings in PNH

A
  • CBC variable
  • retic variable
  • peripheral smear is non-diagnostic
  • direct coombs neg
  • urine hemosiderine elevated
  • serum LDH elevated
  • iron def anemia
  • MUST get flow cytometry*
  • bone marrow bx
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

essentials of PNH dx

A
  • episodic hemoglobinuria
  • thrombosis common
  • suspect in confusing case of hemolytic anemia or pancytopenia
  • flow cytometry shows def of CD55 and CD59
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

treatment of mild PNH

A
  • no intervention required
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

treatment of severe PNH

A
  • transfusion needed
  • eculizumab therapy- improved QOL, reduced hemolysis
  • allogenic SCT
  • treat iron def anemia if indicated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

G6PD deficiency

A
  • def in G6PH enzyme
  • x linked recessive
  • RBCs cant handle oxidative stress -> episodic hemolysis
  • hemoglobin denatures under these conditions
  • extravasc hemolysis in spleen
  • different variants exist in def populations
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

beneficial part of G6PD

A
  • protection from malaria
  • less CAD
  • possibly fewer cancers
  • greater longevity
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

si/sx of G6PD def

A
  • usu healthy
  • no splenomegaly
  • suspect when hemolytic anemia
  • worse with fava beans
23
Q

lab findings with G6PD def

A
  • hemolytic labs appear normal between episodes
  • hgb < 8
  • increased retic count
  • increased serum indirect bili
  • peripheral smear shows bite cells, blisters
  • heinz bodies
  • decreased G6PD enzyme assay 6-8 weeks after hemolysis
24
Q

essentials of G6PD dx

A
  • x linked recessive disorder
  • common in AA men
  • episodic hemolysis in response to oxidant drugs or infections
  • bite cells and blister cells on periph smear
  • reduced level of G6PD btwn hemolytic episodes
25
Q

treatment for G6PD def

A
  • episodes self limited
  • avoid known oxidant drugs
  • pregnant/ nursing- avoid drugs with oxidant potential
  • transfusion if severe epoisode
  • avoid fava beans
  • avoid dapsone*
26
Q

autoimmune hemolytic anemia (AIHA)

A
  • autoab attacking RBCs
  • classified as warm or cold
  • anemia onset can be rapid and life threatening
  • results in spherocytic cells
  • cells get hemolyzed by spleen, if bad enough et hemolyzed by liver
27
Q

what are spherocytic cells

A
  • decreased deformability -> inability to pass through spleen
  • decreased surface area
  • increased fragility
28
Q

si/sx of AIHA

A
  • fatigue
  • dyspnea
  • angina pectoris, possible HF
  • jaundice
  • splenomegaly
29
Q

lab findings

A
  • anemia varies
  • can be severe with hc < 10%
  • increased retic count
  • peripheral smear: spherocytes, NRBCs
  • direct coombs pos*
  • indirect coombs test pos or neg
  • decreased haptoglobin
  • increased direct bili
30
Q

essentials of dx for AIHA

A
  • acquired hemolytic anemia d/t IgG autoab
  • spherocytes and retic on peripheral smear
  • pos coombs
31
Q

treatment of AIHA

A
  • prednisone
  • transfusions- may be difficult to cross match d/t ab
  • splenectomy
  • therpaeutic plasma pheresis
  • refer to hematology
  • sx pts or rapidly falling hbg should get referred
32
Q

cold agglutinin disease

A
  • hemolytic anemia d/t IgM ab
  • coombs test + IgM ab
  • cold ab react with RBCs peripherally
33
Q

what can cause cold agglutinin disease

A
  • waldenstrom
  • macroglobulinemia
  • lymphoma
  • CLL
  • post mycoplasma pneumonia
34
Q

si/ sx of cold agglutinin disease

A
  • sx related to RBC agglutinationon expsoure to cold
  • mottled or numb fingers, toes, ears
  • acrocyanosis
  • episodic LBP
  • dark colored urine
  • hemoglobinuria on exposure to cold
35
Q

essentials for dx of cold agglutinin disease

A
  • increased retic on blood smear
  • coombs pos test for compliment only, neg ab
  • pos cold agglutinin titer
36
Q

treatment for cold agglutinin disease

A
  • avoid exposure to cold
  • rituximab or immunosuppressive therapy
  • if transfusing use in line blood warmer
37
Q

sickle cell disease

A
  • autosomal recessive disorder
  • abnormal hemoglobin
  • predominantly in AA
  • causes chronic hemolytic anemia
  • life span of 40-50 years
38
Q

sickle cell trait

A
  • one good and one bad gene
  • hematologically normal
  • normal life span
39
Q

RBC components in sickle cell

A
  • hemoglobin S (HbS)
  • substitution of valine for glutamic acid
  • poorly soluble when deoxygenated
40
Q

how does sickling occur

A
  • HbS is unstable
  • sickles with deoxygenation
  • undergo hemolysis
  • lifespan of 10-20 days
  • sickle cells can occlude microvasc
41
Q

stressors that can lead to sickle cell crisis

A
  • anything that causes oxidative stress
  • infection
  • hypoxia
  • dehydration
  • acidosis
  • extreme exercise
  • ETOH
  • pregnancy
  • cold weather
42
Q

what is the hallmark sx of sickle cell anemia

A
  • pain due to occlusion
43
Q

si/sx of sickle cell

A
  • pain*
  • anemia, jaundice
  • pigmented gallstones
  • early splenomegaly
  • poor healing ulcers over tibia
  • life threatening anemia
  • chest pain
  • stroke
  • priapism
  • retinopathy
44
Q

types of sickle cell crises

A
  • vaso-occlusive
  • hematologic
  • infection
45
Q

exam findings in sickle cell disease

A
  • dactylitis
  • chronically ill, jaundiced
  • hepatomegaly
  • spleen not palpable
  • cardiomegaly
  • hyperdynamic precordium and systolic murmurs
  • non healing ulcers
  • retinopathy
46
Q

complications of sickle cell disease

A
  • retinopathy
  • pulm htn- poor prognosis
  • delayed puberty
  • infex
  • bone necrosis
  • cardiomegaly
  • chlelithiasis
47
Q

lab studies for sickle cell anemia

A
  • hct 20-30%
  • WBC and platelets elevated
  • blood smear: irreversibly sickled cells, reticulocytes, howell-jolly bodies, target cells
  • HgS screening
  • Hgb electrophoresis
  • increased indirect bili
48
Q

essentials of dx of sickle cell anemia

A
  • recurrent pain
    • family hx and lifelong hx of hemolytic anemia
  • irreversibly sickled cells on smear
  • HbS on electrophoresis
49
Q

treatment for sickle cell anemia

A
  • supoprtive care
  • folic acid PO
  • pneumococcal vaccine
  • avoid precipitating factors
  • exchange transfusion if indicated
  • hydroxyurea if severe
  • omgea 3 fatty acid supplementation
  • allogenic HSCT
50
Q

treatment in acute crisis of sickle cell disease

A
  • generous analgesia*
  • transfusion for aplastic or hemolytic crisis
  • ID precipitating factors and treat/ avoid
  • keep well hydrated, O2 if hypoxic
  • abx, VTE ppx
51
Q

sickle cell trait

A
  • heterozygous genotype
  • AS instead of SS
  • hematologically normal
  • screening for sickle cell hg +
  • no tx needed
52
Q

clinical risk sof sickle cell trait

A
  • sudden cardiac death or rhabdo with vigerous exs, esp at night
  • increased risk VTE
  • microscopic and gross hematuria
  • hyposthenuria
  • possible CKD
53
Q

when to refer for sickle cell anemia

A
  • all pts referred to hematology
  • all pts referred to comprehensive sickle cell center
  • prenatal dx avail for couples at risk
54
Q

benefits of sickle cell anemia

A
  • resistance to malaria
  • cells have shorter life
  • eliminated in spleen
  • cell membrane stretched by its shape, becomes porous and leaks nutrients like K
  • parasite needs those nutrients for survival