Exam 4: Anemia Flashcards

1
Q

The following lab findings are indicative of what type of anemia?

Reticulocytosis

Increased LDH

Increased indirect bilirubin

decreased haptoglobin

schistocytes on peripheral smear

A

Hemolytic anemia

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2
Q

why do you see increased reticulocytosis in hemolytic anemia?

A

bone marrow is trying to compensate for increased RBC destruction by releasing immature reticulocytes

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3
Q

What causes the increased serum LDH in hemolytic anemia?

A

LDH is found in RBCs, increased destruction of RBCs means increased LDH in serum

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4
Q

what causes the dark urine in hemolytic anemia?

A

increased urine hemosiderin, increased direct bilirubin

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5
Q

why does indirect bilirubin increase in hemolytic anemia?

A

increased RBC lysis overwhelms liver’s conjugation ability.

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6
Q

what sign may manifest as a result of increased serum indirect bilirubin?

A

jaundice

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7
Q

Why do you see decreased serum haptoglobin in intravascular hemolytic anemias?

A

serum haptoglobin binds to free Hb leading to low haptoglobin due to increased free Hb.

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8
Q

What should you expect to see on peripheral smear in hemolytic anemias?

A

+ schistocytes
+ reticulocytes (diff. color)
nucleated RBCs

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9
Q

What lab findings would indicate hemolytic anemia?

A

reticulocytosis

Increased LDH

Increased Indirect Bilirubin

Decreased Haptoglobin

+schistocytes and nucleated RBCs on peripheral smear

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10
Q

what two molecules store iron?

A

Ferritin and hemosiderin (macrophages)

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11
Q

Match the following lab values with either:

A. Iron Deficiency Anemia
B. Anemia of Chronic Disease

  1. Decreased serum Fe, Decreased Ferritin, Increased TIBC
  2. Decreased serum Fe, Increased Ferritin, Decreased TIBC
A
  1. = A

2. = B

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12
Q

A pt. presents c the following laboratory results.

MCV 85
Serum Fe: Decreased
Ferritin: Increased
TIBC: Decreased

What type of anemia do you suspect?

A

anemia of chronic disease

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13
Q

What comorbid conditions do you expect to see in a patient presenting with the following lab results…

Decreased Serum Fe
Increased Ferritin
Decreased TIBC
MCV Normal

A

Anemia of chronic disease:
Chronically ill patients

inflammatory disease, rheum disorder, cancer, chronic infection, organ failure

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14
Q

how would you treat a patient presenting with anemia of chronic disease?

A

treat the underlying cause of anemia +/- EPO

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15
Q

A pt. presents c the following laboratory results.

Iron Studies–

Serum Fe: normal/increased
Ferritin: normal/increased
TIBC: normal/decreased

CBC---
MCV 65
RBC: normal/increased
RDW: Normal
Retic: elevated

What type of anemia do you suspect? What is the hallmark that lead you to that Dx?

A

Thalassemia. As evidenced by:

-microcytic anemia (low MCV) with normal RDW, increased RBC and normal serum Fe.

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16
Q

What would you expect to see on peripheral smear in a pt. with one of the thalassemia

A

Target cells, tear drop cells, microcytosis, hypochromia

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17
Q

what additional test outside of peripheral smear, CBC, and iron studies can help diagnose and detect the type of thalassemia present?

A

Hb electrophoresis

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18
Q

What is the normal genotype for hemoglobin a?

A

aa/aa + B/B

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19
Q

What is the genotype in silent carrier alpha thalassemia?

A

aa/a- , 1 deletion

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20
Q

what is the genotype in alpha thalassemia minor

A

aa/– or a-/a- , 2 deletions

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21
Q

What is the genotype in alpha thalassemia intermediate?

A

a-/– , 3 deletions

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22
Q

what is the genotype in alpha thalassemia major/hydrops fetalis?

A

–/– , 4 deletions, fatal in utero

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23
Q

B-Thalassemia trait is the result of what genotype?

A

dysfunction in one B-globin chain

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24
Q

Thalassemia intermedia presents with what condition?

A

chronic hemolytic anemia

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25
Q

B-Thalassemia Major presents with what genotype?

A

dysfunction in both B-globin chains

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26
Q

What is the result of B-Thalassemia Major in hemoglobin?

A

excess alpha chains are unable to form tetramers leading to ineffective erythropoiesis

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27
Q

B-Thalassemia major presents with what kind of anemia?

A

severe, transfusion dependent hemolytic anemia

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28
Q

With hemoglobin electrophoresis, bThal presents with what?

A

increased hbA2 and HbF

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29
Q

A patient presents with the following lab results:

Low MCV
Normal RDW
Normal Ferritin
Normal/increased Serum Fe

Electrophoresis: Increased HbA2 and HbF

What tx should be considered?

A

folic acid supplementation

regular transfusions and chelation therapy

Hematopoietic cell transplant

Genetic counseling

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30
Q

A pt. from a mediterranean descent presents with normal RDW, low MCV, increased serum Fe and increased RBCs.

What should you suspect and why?

A

B-Thal, MC in mediterranean populations

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31
Q

A patient presents to the clinic with pallor, fatigue, syncope and tachycardia. Pt. admits to pica, and exhibits the following on PE:

Atrophic glossitis
Angular Cheilitis
Koilonychia
Dysphagia c esophageal webs

What should these signs make you suspicious of?

A

Iron Deficiency Anemia

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32
Q

A patient presenting with pallor, weakness, dyspnea and palpitations presents with the following laboratory results.

CBC---
RBC: Decreased
MCV: normal/decreased
RDW: increased
retic: low to normal

Iron Studies:
Ferritin: Decreased
Serum Fe: Decreased
TIBC: Increased

What do you suspect and what are the 3 most common causes?

A
  1. Dx of IDA

2. Caused by hemorrhage, decreased Fe intake, decreased Fe absorption 2ry to celiac, h. pylori, bariatric surg.

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33
Q

A patient presents with the following laboratory studies…

Ferritin: Decreased
Serum Fe: Decreased
TIBC: Increased

What treatments should be considered?

A

Oral ferrous sulfate 325 mg PO QD-TID on empty stomach.

Consider blood transfusions or parenteral iron

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34
Q

What is an appropriate response to PO iron supplementation?

A

increasing Hb at rate of 2-4 every 3 weeks.

Tx continued 3-6 months after anemia has corrected to replenish stores

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35
Q

What is the pathology of sideroblastic anemia?

A

Abnormal RBC iron metabolism leading to diminished heme synthesis and iron accumulation in the cells

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36
Q

What is the common etiology of sideroblastic anemia?

A

Acquired via:
chronic etoh
medications
copper deficiency

or

Congenital via:
autosomal recessive X-linked

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37
Q

what etiology of sideroblastic anemia is more common in adults, acquired or hereditary?

A

acquired

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38
Q

A pt. presents with sx of anemia. Laboratory studies show the following:

MCV: normal
RDW: elevated
Retic: normal/low
Ferritin: normal high

bone marrow aspirate: Ring Sideroblasts

Peripheral smear: siderocytes with pappenheimer bodies

What do you suspect?

A

sideroblastic anemia

39
Q

what are two hallmark diagnostic findings for sideroblastic anemia

A

ring sideroblasts on bone marrow aspirate

siderocytes with pappenheimer bodies on peripheral smear

40
Q

A pt. presents with ring sideroblasts and pappenheimer bodies on bone marrow aspirate and peripheral smear respectively… what tx do you consider?

A

refer to hematology and treat underlying cause

discontinue offending drugs/toxic agents if necessary

pyridoxine supplementation and transfusion PRN

41
Q

what causes the decrease in serum Fe in anemia of chronic disease?

A

hepcidin causes trapping of iron in macrophages and decreased iron absorption in gut.

42
Q

What differences should you expect to see on peripheral smear and bone marrow aspiration of b12 deficiency and folate deficiency?

A

none!

43
Q

A pt. presents to the clinic c sxs of anemia, glossitis, and GI discomfort alongside chronic alcoholism.

What should you immediately suspect and what are other common etiologies of this disorder?

A

Folic Acid Deficiency megaloblastic macrocytic anemia.

Also caused by

Hemodialysis

Elderly

end term pregnancy

anticonvulsant therapy, MTX, TMP-SMZ

hemolytic anemia

44
Q

You suspect your alcoholic pt. presenting with glossitis, GI sxs and pallor has a folic acid deficiency. You order a CBC and blood smear showing the following…

MCV 115
Peripheral Smear: hypersegmented neutrophils

What test do you want to order next and what do you expect to find?

A

Serum Folate: Increased

Serum homocysteine: increased

Serum methylmalonic acid: normal

Serum B12: normal

45
Q

How do you treat your patient with folic acid deficiency?

A

1mg folic acid PO QD with food

rule out b12 deficiency

46
Q

What is the most common cause of vitamin B12 deficiency/

A

pernicious anemia

47
Q

Besides PA, what are other common causes of vitamin b12 deficiency?

A

decreased intake 2ry to vegan diet

Metformin, H2 antagonists, PPIs

Malabsorption in elderly

Gastric/illeal disease or surgery

48
Q

This disease is autoimmune mediate destruction of the parietal cells. What is the disease and what occurs as a result of parietal cell destruction?

A

Pernicious anemia.

Parietal cell destruction means decreased intrinsic factor secretion

49
Q

What results from decreased intrinsic factor secretion?

A

decreased gastric acid secretion, and Pernicious anemia/vit. B12 deficiency

Increased risk of gastric cancer

50
Q

A patient presents to the clinic with the following sxs and findings on PE:

Skin:
(+) pallor of skin and conjunctiva

HEENT:
(+) glossitis, stomatitis

GI:
(+) mild GI sx, diarrhea

Neuro:
(-) vibratory and position sense
(+) ataxia, stocking paresthesia, confusion

What are you suspicious of and what key findings clued you in?

A

Dx of vit. b12 deficiency due to + Neuro sxs

51
Q

Aa patient with CNS sxs and anemia sxs presents to the clinic. CBC, peripheral smear indicate the following:

MCV increased
WBC increased
Platelets increased

Peripheral smear:
hypersegmented neutrophils
+anisocytosis, poikilocytosis, macro ovalocytes

What is your Dx and what helped you differentiate it from other disorders?

A

b12 deficiency, + hypersegmented neutrophils, macroovalocytosis

52
Q

You suspect a patient has megaloblastic macrocytic anemia due to vitamin b12 deficiency. Besides CBC, smear, and aspiration, what tests do you want and what do you expect to find?

A
  1. Serum b12: decreased
  2. Serum methylmolonic acid: increased
  3. Serum homocysteine: increased
  4. Folate: normal
53
Q

if you suspect pernicious anemia, what additional tests should be ordered?

A

(+) Intrinsic Factor Ab
(+) parietal cell Ab
Increased Gastrin level

(+) Schilling test

54
Q

how do you treat vitamin v12 deficiency with supplementation

A
  1. Parenteral B12

Vitamin b12 1000 microgram IM/SQ QD for 1 week

Then once weekly for one month, then monthly for life

55
Q

What is important to monitor when treating vitamin b12 deficiency?

A

potassium… new cell formation can lead to consumption of potassium

56
Q

In addition to typical anemia sxs, what do you expect to see with hemolytic anemias?

A

Jaundice, dark urine, gallstones

57
Q

A patient presents to the clinic with back pain, jaundice, splenomegaly and anemia sxs. The patient just finished a course of TMP-SMZ.

What type of hemolytic anemia do you suspect?

A

G6PD deficiency

58
Q

Your anemia patient shows the following lab results:

Peripheral smear: + heinz bodies, bite cells

MCV: 87
retic: increased
indirect bilirubin: increased
haptoglobin: decreased

What type of anemia is this and what was a hallmark?

A

G6PD deficiency due to:
(+) heinz bodies
Elevated indirect bili, retic

59
Q

why are female carriers rarely affected by G6PD deficiency?

A

it is an X-linked recessive disorder

60
Q

what are common causes of oxidative stress that can cause G6PD crisis?

A

sulfa, antimalarials, aspirin

infx

fava beans

61
Q

how should you treat your patient with hemolytic crisis due to G6PD deficiency?

A

self-limited, avoid triggers

62
Q

episodic hx of hemolytic crisis

preceded by sulfa, fava, infx

Dx this…

A

G6PDD

63
Q

sickle cells on peripheral smear

+ HbS on electrophoresis

Dx this…

A

sickle cell anemia

64
Q

microspherocytes

Coombs NEGATIVE

(+) osmotic fragility

Dx this…

A

hereditary spherocytosis

65
Q

(+) microspherocytes

(+) Coombs

Dx this…

A

autoimmune hemolytic anemia

66
Q

dark urine worse in the am

Dx this…

A

PNH

67
Q

hereditary spherocytosis is characterized by what type of inheritance pattern?

A

autosomal dominant intrinsic anemia

68
Q

A well appearing pt. presents with mild jaundice and icterus. PE shows splenomegaly. Pt. complains of abdominal pain.

US + for gallstones

Labs show the following:

Osmotic fragility test: POS
Coombs: NEG

Peripheral smear: POS spherocytes, hyperchromic microcytosis

what type of hemolytic anemia do you suspect? What is the tx of choice?

A

Hereditary spherocytosis

Tx with splenectomy

69
Q

When treating hereditary spherocytosis with splenectomy, what should you consider?

A

delay splenectomy until adulthood and supplement with Folate until then

70
Q

what is the hereditary pathway of sickle cell disease?

A

autosomal recessive

71
Q

What is the genotype of sickle cell disease?

A

Hb SS

72
Q

what is the genotype of sickle cell trait?

A

HbS + HbA

73
Q

A 6 month old patient presents to the clinic with delayed growth and dactylitis. What is your presumptive Dx and what precipitated the onset of these sxs?

A

Common early signs and sx of sickle cell.

Occurs when fetal hb (HbF) changes to adult HbS

74
Q

You have a pediatric patient whom you’ve just diagnosed with sickle cell disease. What patient education are you providing to help manage the disease?

A

Pt. will have increased susceptibility to infections due to functional asplenia.

Aplastic crisis is associated with parvovirus B19

Sxs precipitated by hypoxic conditions like dehydration, high altitude, intense exercise

75
Q

Aplastic crisis, pain crisis, osteonecrosis, acute chest syndrome, CVA/MI and other infarcts are common with what disease?

A

sickle cell anemia

76
Q

A patient presents with severe pain after football practice. The labs are as follows:

HCT: Decrease
Retic: Increased
MCV: 90

What tests do you want to order, what do you suspect will be the result, and what is your Dx?

A

Hb Electrophoresis to reveal HbS

Peripheral smear: sickled RBCs, nucleated RBCs, target cells, Howell-Jolly bodies

Dx of sickle cell disease

77
Q

What is the gold standard test to diagnose sickle cell disease?

A

Hb Electrophoresis revealing Hb S

78
Q

How do you treat sickle cell disease?

A

avoidance of triggers

Analgesics, fluid, O2 in crisis

Transfusion if needed

Hydroxyuria

Bone marrow transplant

79
Q

This disease is caused by autoAbs adhering to RBCs to induce hemolysis and complement activation.

A

Autoimmune hemolytic anemia

80
Q

What differentiates primary and secondary autoimmune hemolytic anemia?

A

primary: no underlying systemic disorder
secondary: identifiable underlying systemic illness

81
Q

“cold” agglutinins illicit what type of response, and are mediated by what antibody?

A

Acute sxs, IgM

82
Q

“warm” agglutinins illicit what type of response and are mediated by what antibody?

A

attack at normal body temperature, IgG

83
Q

What are the common causes for autoimmune hemolytic anemia?

A

SLE, RA

CLL

Mycoblasma, EBV, HIV infx

immunodeficiency

blood transfusion

drugs

84
Q

A patient presents with anemia-like sxs, LAD, jaundice, splenomegaly and is complaining of hemoglobinuria. On physical exam you notice acrocyanosis.

What is your presumptive dx? What test would confirm this?

A

Autoimmune hemolytic anemia.

(+) Coombs

85
Q

What laboratory test distinguishes autoimmune hemolytic anemia from Hereditary spherocytosis?

A

(+) Coombs indicates AIHA

(-) Coombs indicates HS

86
Q

What do you expect to find on peripheral smear of a patient with AIHA?

A

polychromasia, sphereocytosis, nucleated RBC

87
Q

How do you treat Warm AIHA?

A

CS, rituximab, splenectomy

88
Q

How do you treat Cold AIHA?

A

avoid cold, Rituxumab, plasmapheresis if refractory

89
Q

The presence of schistocytes due to mechanical heart valve intravascular hemolysis is called what?

A

Fragmentation Syndrome

90
Q

A patient who received a blood transfusion 4 hours ago is experiencing fever, allergic reactions and hemolytic sxs. What is causing this?

A

hemolytic transfusion reaction

91
Q

This disease is a rare, acquired stem cell mutation characterized by complement mediated RBC lysis. What disease is this?

A

Paroxysmal Nocturnal Hemoglobinuria

92
Q

A patient is presenting w/ jaundice, anemia sxs, and cola colored urine in the AM.

CBC reveals pancytopenia.

What disease do you suspect, and what is a worrisome effect?

A

Paroxysmal Nocturnal Hemoglobinuria. Concern for venous thrombosis

93
Q

You suspect your patient has PNH. What tests do you order to confirm your dx?

A

flow cytometry, osmotic fragility, (-) Coombs

94
Q

How do you treat PNH?

A

monoclonal antibody against complement C5, steroids, stem cell transplant.