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Senior - Heme-Onc > Lecture 4 - Normocytic Anemia > Flashcards

Lecture 4 - Normocytic Anemia Flashcards

1
Q

DDX for normocytic anemai?

A 
Ancemia of chronic disease
Acute blood loss
Multifactorial anemia
Renal-failure associated anemia
Aplastic anemia
Hemolysis (hemolytic anemia)
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2
Q

2nd MC anemia?

A

Anemia of chronic disease (ACD)

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3
Q

Lab description of ACD?

A

Normochromic
Normocytic
Hypoproliferative

May be microcytic (late sign)

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4
Q

With ACD what determines the severity and characteristics of the anemia?

A

The underlying disease

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5
Q

What is the key pathophysiologic principle of ACD?

A

Disturbance in iron homeostasis mediated by chronic inflammation

Hepcidin released from liver in presence if IL-6

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6
Q

Why does the inflammation process cause anemia?

A

Inflammatory process leads to decreased iron uptake from GI tract and retention of iron w/in the macrophages as ferritin

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7
Q

Inflammatory processes cause a 2 pronged attack, what is it?

A
  1. Decreased iron availability in the bone marrow leads to reduced RBC production-hypoproliferation
  2. Reduced RBC survival (increased destruction)
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8
Q

How sever is the anemia with ACD?

A

Usually its mild but is often associated with other anemic processes

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9
Q

Lab findings for ACD?

A 
Normocytic: MCV 80-100
Low iron
Low transferrin
Low TIBC
HIGH ferritin
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10
Q

TX for ACD?

A

Treat the disease
Blood transfusion
EPO if symptomatic and Hb <10g/dL

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11
Q

Iron supplementation for ACD?

A

Not generally recommended if no iron deficiency

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12
Q

Charts on

A

Slides 18 and 19, maybe give them a look

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13
Q

What type of anemia is acute blood loss?

A

Normocytic anemia

- youre blood is fine you just dont have enough of it

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14
Q

With a hemorrhage pt you cannot trust?

A

CBC, they are getting IV fluids and blood products as part of their therapy and it will throw off their stuff

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15
Q

What is multifactorial anemia?

A

You have multiple causes for your anemia

Often Iron and B12 or iron and acd etc

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16
Q

Who gets renal failure associated anemia?

A

Prolonged renal failure:
- GFR < 30mL/min

It is a normocytic anemia

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17
Q

Erythropoietin, what is it good for?

A

Absolutely nothing!

Probably inaccurate however we cant count on it for diagnosis of renal failure associated anemia as it looks normal until Hb < 10

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18
Q

What is the tx for renal failure associated anemia?

A

Chronic synthetic erythropoietin agents (ESAs)

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19
Q

Hypoproliferative anemia is a prominent feature in?

A

Hematologic diseases described as bone marrow failure states

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20
Q

What is aplastic anemia?

A

It is a condition Bone marrow failure caused by:

Abnormal expression of stem cells leading to hypoplastic bone marrow.

Pancytopenia ensues

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21
Q

Primary and secondary aplastic anemia?

A

Primary:
-MC IgG autoantibody directed against the stem cell

Secondary:

  • inherited (fanconi’s)
  • acquired (chemo, radiation et)
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22
Q

Bone marrow disorders that cause pancytopenia?

A
  • Aplastic anemia
  • Myelodysplasia
  • Acute leukemia
  • Myelofibrosis
  • Infiltrative disease
    • (lymphoma, myeloma, carcinoma)
  • Megalobastic anemia
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23
Q

Non-marrow disorders that cause pancytopenia?

A
  • hypersplenism

- SLE

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24
Q

Clinical features of aplastic anemia?

A
  • Anemia: Weakness, fatigue, pallor
  • neutropenia - infection
  • thrombocytopenia - bleeding
  • P/E (pallor, purpura, petechiae)
  • bimodal distribution (15 -25 yo then 60+ yo)
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25
Q

Labs for aplastic anemia?

A

Severe anemia
Pancytopenia (hallmark)
Decreased reticulocytes
Normal RBC

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26
Q

How is the diagnosis for aplastic anemia made?

A

CBC - pancytopenia

Bone marrow bx - hypocellular bone marrow

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27
Q

What is fanconi’s anemia?

A

Inherited form of aplastic anemia

Highly variable phenotypic expression of autosomal recessive trait or x-linked disorder

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28
Q

Fanconi’s anemia is characterized by?

A

Pancytopenia

Congenital malformations

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29
Q

Major causes of aplastic anemias

A

Slide 29, give it a gander

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30
Q

Tx for aplastic anemia?

A

Varies by age

20 y/o: bone marrow transplant (curative)

> 45y/o: immunosuppression, let body fix itself

20-45y/o: shooter preference

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31
Q

Prognosis for aplastic anemia?

A

<50y/o: 70% 5 yr survival
>70y/o: 50% 5 yr survival

Severe unresponsive aplastic anemia have 1 yr untreated mortality rate of 70%

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32
Q

What are hemolytic anemias?

A

Condition in which RBCs are destroyed and removed from circulation
- result is fewer mature RBCs which causes anemia

Broad range of etiologies

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33
Q

Classifications for hemolytic anemias?

A

Intrinsic: defect in RBC (hereditary)

Extrinsic: external to RBC

34
Q

Lab descriptions of hemolytic anemias?

A

Peripheral Blood Smear

  • spherocytes - round, lack concavity and central pallor
  • schistocytosis - fragmented/destroyed rbc
  • helmet cells
  • target cells
  • reticulocytis
35
Q

Labs for hemolytic anemias?

A 
Haptoglobin : L
Indirect bilirubin : H
Hemoglobinuria : H
LDH : H
Reticulocytes : L/Normal
36
Q

I am an acquired clonal stem cell D/o, what am I?

My MC mutation is PIG-A gene

A

Paroxysmal nocturnal hemoglobinuria (PNH)

37
Q

What is a PIG-A gene?

A

Gene responsible for some stuff… CD55 and CD59…

Result is RBC are susceptible to complement-mediated hemolysis

38
Q

Clinical manifestation of PNH?

A

Episodic dark cola-colored urine (wake up pee)

Prone to thrombosis (veins > arteries)

Stem cell disorder

39
Q

What conditions are associated with PNH?

A

It is a stem cell d/o so:

Aplastic anemia
Myelodysplasia
Acute myeloid leukemia

40
Q

Hallmarks (Labs) for PNH?

A

LDH - H
Urine hemosiderin - +
Haptoglogin - L
iron def

41
Q

Best screening test for PNH?

A

Flow cytometry

- def of CD55 and CD59 on RBCs

42
Q

Key words for PNH diagnosis?

A
  • Episodic hemoglobinuria
  • Hemolytic anemia
  • Thrombosis (hypercoagulable state)
  • Flow cytometry (screening)
43
Q

Tx of Paroxysmal Nocturnal Hemoglobinuria

A

Referral

Eculizumab - reduce hemolysis
Anticoagulants
Iron 
Bone marrow transplant 
Prednisone
44
Q

MC hemolytic anemia to to RBC membrane defect?

A

Hereditary spherocytosis

45
Q

What is going on with hereditary spherocytosis?

A

an abnormailty in spectrein or actin

Basically the RBC membrane doesnt flex like it should so they are rigid and dont pass through small fenestrations in spleen

Leads to badness (i mean hemolysis)

46
Q

What type of anemia does hereditary spherocytosis cause?

A

Chronic hemolytic anemia

47
Q

Clinical findings for hereditary spherocytosis?

A

Autosomal dominant disorder (fam hx)

Dx’d in childhood

Chronic hemolysis causes

  • jaundice,
  • splenomegaly
  • pigmented gallstones
48
Q

Labs for hereditary spherocytosis?

A

Microcytosis or normocytic and increase MCHC

Reticulocytis

Spherocytes

Neg coomgs (antibody test)

Osmotic fragility test - increased

49
Q

What is the only condition that has increased MCHC?

A

Hereditary spherocytois

so if you see elevated MCHC pick this one!!!

50
Q

Hereditary spherocytosis is intrinsic or extrinsic?

A

Intrinsic (its the RBC membrane that is fucked)

51
Q

Tx for hereditary spherocytosis?

A

Supportive care
Folic acid 1mg/d
Splenectomy (that’ll teach the spleen)

52
Q

This d/o causes acute hemolytic anemia with hemoglobinuria when taking the anti-malaria drug primaquine

A

G6PD deficiency

53
Q

MC enzymatic d/o of RBC?

A

G6PD

X-linked enzyme defect

54
Q

What are heinz bodies?

A

Oxidized Hgb denatures to form precipitants called heinz bodies

These damage RBC membrane and the spleen takes them out

Seen with G6PD

55
Q

Pathophysiology of G6PD?

A

Oxidative stress
- cellular damage occurs from radical oxygen species that are not fully neutralized by anti-oxidants

G6PD folks cant handle the stress

56
Q

Drugs that affect G6PD?

A

Antimalarials
Sulfonamides
Nitrofuratonin
And some misc stuff

57
Q

Tx for G6PD?

A

Avoid that shit

58
Q

Who gets G6PD deficiency?

A

10-15% of AA males

59
Q

Labs for G6PD?

A

Normal between episodes

During episodes

  • reticulocytosis
  • indirect bilirubin
  • bite cells
  • heinz bodies
60
Q

What is sickle cell anemia?

A

Autosomal recessive disorder
Characterized by point mutation of beta globin chain gene

Sickling

61
Q

What causes hemolytic crisis for sickle cells pts?

A

Splenic sequestration of sickled cells

62
Q

Epidemiology of Sickle Cell Anemia?

A

8% of AA are carriers, 1 in 400 has Sickle Cell Disease

63
Q

Clinical findings of Sickle cell anemia?

A

Usually Show up in 1st year of life

Chronic hemolytic anemia
Pain (acute vaso-occlusion from clusters)

Acute chest syndrome

64
Q

What is acute chest syndrome?

A

Fevers
Chest pain
Hypoxemia
Pulmonary infiltrates

65
Q

Howel-jolly bodies

A

Hallmarks of hyposplenism due to auto-infarct and vaso occlusion (sickle cell)

(Nuclear remnants w/in RBCs that are usually removed by spleen and target cells)

Thats all cool, it looks like some shit let on the RBC when you look at a microscope

66
Q

Lab findings for sickle cell

A

Chronic hemolytic anemia
howell-jolly bodies
Chronic reticulocytosis

67
Q

How is sickle cell confirmed?

A

Hb electrophoresis

68
Q

Tx for sickle cell?

A

Supportive care
Hydroxyurea
Transfusion
Pneumococcal vacc

69
Q

Proposed sickle cell treatments?

A

L-glutamine
Rivipansel
Crizanlizumab

See slide 60-62 if you care, it wont be a question b/c they are still in trials

70
Q

Hospital therapy for sickle cell crisis?

A 
IV
DVT prophylaxis
Diphenhydramine
NSAIDs
Opioid therapy
71
Q

Autoimmune hemolytic aneima (AIHA)

A

Acquired autoantibodies to RBC

It is an extrinsic defect

72
Q

MC AIHA?

A

“Warm” AIHA

IgG antibodies react at normal body temps and destroy RBCs

73
Q

Cold AIHA?

A

Aka cold agglutinin disease

IgM antibodies react at reduced body temps

74
Q

Clinical feature of autoimmune hemolytic anemia?

A
  • Episodic associated w physiologic stress
  • Rapid onset anemia
  • fatigue, angina, dypsnea, palpitations
  • pain w eating/drinking cold stuff
  • spherocytosis
    + COOMBS test
75
Q

Why does AIHA have positive Coombs?

A

B/c its autoimmune and thats what Coombs is fore

76
Q

Warm AIHA tx?

A

Refer to hematology
Immunosuppression
Splenectomy
Transfusions (complicated)

77
Q

Cold AIHA tx?

A

Refer to hematology
Supportive
Avoid cold
Rituximab

78
Q

What is microangiopathic hemolytic anemias (MAHA)?

A

Group of d/o

Intravascular hemolysis due to RBC fragmentation (traumatic destruction)

79
Q

Hallmark of MAHA?

A

Fragmented cells on PBS

  • schistocytosis
  • helmet cells
80
Q

Pts w hemolytic anemia are more prone to?

A

Infections liek parvovirus and strep

Infections may trigger hymolysis due to “unique mechanisms” i.e. magic

81
Q

Malaria

A

JK he just had a title slide of this, no info

82
Q

They say blood is thicker than water

A

So what? So is toothpaste

Senior - Heme-Onc (6 decks)

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