Exam 4: Anemia Flashcards

(94 cards)

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
B-Thalassemia Major presents with what genotype?
dysfunction in both B-globin chains
26
What is the result of B-Thalassemia Major in hemoglobin?
excess alpha chains are unable to form tetramers leading to ineffective erythropoiesis
27
B-Thalassemia major presents with what kind of anemia?
severe, transfusion dependent hemolytic anemia
28
With hemoglobin electrophoresis, bThal presents with what?
increased hbA2 and HbF
29
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?
folic acid supplementation regular transfusions and chelation therapy Hematopoietic cell transplant Genetic counseling
30
A pt. from a mediterranean descent presents with normal RDW, low MCV, increased serum Fe and increased RBCs. What should you suspect and why?
B-Thal, MC in mediterranean populations
31
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?
Iron Deficiency Anemia
32
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?
1. Dx of IDA | 2. Caused by hemorrhage, decreased Fe intake, decreased Fe absorption 2ry to celiac, h. pylori, bariatric surg.
33
A patient presents with the following laboratory studies... Ferritin: Decreased Serum Fe: Decreased TIBC: Increased What treatments should be considered?
Oral ferrous sulfate 325 mg PO QD-TID on empty stomach. Consider blood transfusions or parenteral iron
34
What is an appropriate response to PO iron supplementation?
increasing Hb at rate of 2-4 every 3 weeks. Tx continued 3-6 months after anemia has corrected to replenish stores
35
What is the pathology of sideroblastic anemia?
Abnormal RBC iron metabolism leading to diminished heme synthesis and iron accumulation in the cells
36
What is the common etiology of sideroblastic anemia?
Acquired via: chronic etoh medications copper deficiency or Congenital via: autosomal recessive X-linked
37
what etiology of sideroblastic anemia is more common in adults, acquired or hereditary?
acquired
38
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?
sideroblastic anemia
39
what are two hallmark diagnostic findings for sideroblastic anemia
ring sideroblasts on bone marrow aspirate siderocytes with pappenheimer bodies on peripheral smear
40
A pt. presents with ring sideroblasts and pappenheimer bodies on bone marrow aspirate and peripheral smear respectively... what tx do you consider?
refer to hematology and treat underlying cause discontinue offending drugs/toxic agents if necessary pyridoxine supplementation and transfusion PRN
41
what causes the decrease in serum Fe in anemia of chronic disease?
hepcidin causes trapping of iron in macrophages and decreased iron absorption in gut.
42
What differences should you expect to see on peripheral smear and bone marrow aspiration of b12 deficiency and folate deficiency?
none!
43
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?
Folic Acid Deficiency megaloblastic macrocytic anemia. Also caused by Hemodialysis Elderly end term pregnancy anticonvulsant therapy, MTX, TMP-SMZ hemolytic anemia
44
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?
Serum Folate: Increased Serum homocysteine: increased Serum methylmalonic acid: normal Serum B12: normal
45
How do you treat your patient with folic acid deficiency?
1mg folic acid PO QD with food rule out b12 deficiency
46
What is the most common cause of vitamin B12 deficiency/
pernicious anemia
47
Besides PA, what are other common causes of vitamin b12 deficiency?
decreased intake 2ry to vegan diet Metformin, H2 antagonists, PPIs Malabsorption in elderly Gastric/illeal disease or surgery
48
This disease is autoimmune mediate destruction of the parietal cells. What is the disease and what occurs as a result of parietal cell destruction?
Pernicious anemia. Parietal cell destruction means decreased intrinsic factor secretion
49
What results from decreased intrinsic factor secretion?
decreased gastric acid secretion, and Pernicious anemia/vit. B12 deficiency Increased risk of gastric cancer
50
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?
Dx of vit. b12 deficiency due to + Neuro sxs
51
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?
b12 deficiency, + hypersegmented neutrophils, macroovalocytosis
52
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?
1. Serum b12: decreased 2. Serum methylmolonic acid: increased 3. Serum homocysteine: increased 4. Folate: normal
53
if you suspect pernicious anemia, what additional tests should be ordered?
(+) Intrinsic Factor Ab (+) parietal cell Ab Increased Gastrin level (+) Schilling test
54
how do you treat vitamin v12 deficiency with supplementation
1. Parenteral B12 Vitamin b12 1000 microgram IM/SQ QD for 1 week Then once weekly for one month, then monthly for life
55
What is important to monitor when treating vitamin b12 deficiency?
potassium... new cell formation can lead to consumption of potassium
56
In addition to typical anemia sxs, what do you expect to see with hemolytic anemias?
Jaundice, dark urine, gallstones
57
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?
G6PD deficiency
58
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?
G6PD deficiency due to: (+) heinz bodies Elevated indirect bili, retic
59
why are female carriers rarely affected by G6PD deficiency?
it is an X-linked recessive disorder
60
what are common causes of oxidative stress that can cause G6PD crisis?
sulfa, antimalarials, aspirin infx fava beans
61
how should you treat your patient with hemolytic crisis due to G6PD deficiency?
self-limited, avoid triggers
62
episodic hx of hemolytic crisis preceded by sulfa, fava, infx Dx this...
G6PDD
63
sickle cells on peripheral smear + HbS on electrophoresis Dx this...
sickle cell anemia
64
microspherocytes Coombs NEGATIVE (+) osmotic fragility Dx this...
hereditary spherocytosis
65
(+) microspherocytes (+) Coombs Dx this...
autoimmune hemolytic anemia
66
dark urine worse in the am Dx this...
PNH
67
hereditary spherocytosis is characterized by what type of inheritance pattern?
autosomal dominant intrinsic anemia
68
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?
Hereditary spherocytosis Tx with splenectomy
69
When treating hereditary spherocytosis with splenectomy, what should you consider?
delay splenectomy until adulthood and supplement with Folate until then
70
what is the hereditary pathway of sickle cell disease?
autosomal recessive
71
What is the genotype of sickle cell disease?
Hb SS
72
what is the genotype of sickle cell trait?
HbS + HbA
73
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?
Common early signs and sx of sickle cell. Occurs when fetal hb (HbF) changes to adult HbS
74
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?
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
Aplastic crisis, pain crisis, osteonecrosis, acute chest syndrome, CVA/MI and other infarcts are common with what disease?
sickle cell anemia
76
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?
Hb Electrophoresis to reveal HbS Peripheral smear: sickled RBCs, nucleated RBCs, target cells, Howell-Jolly bodies Dx of sickle cell disease
77
What is the gold standard test to diagnose sickle cell disease?
Hb Electrophoresis revealing Hb S
78
How do you treat sickle cell disease?
avoidance of triggers Analgesics, fluid, O2 in crisis Transfusion if needed Hydroxyuria Bone marrow transplant
79
This disease is caused by autoAbs adhering to RBCs to induce hemolysis and complement activation.
Autoimmune hemolytic anemia
80
What differentiates primary and secondary autoimmune hemolytic anemia?
primary: no underlying systemic disorder secondary: identifiable underlying systemic illness
81
"cold" agglutinins illicit what type of response, and are mediated by what antibody?
Acute sxs, IgM
82
"warm" agglutinins illicit what type of response and are mediated by what antibody?
attack at normal body temperature, IgG
83
What are the common causes for autoimmune hemolytic anemia?
SLE, RA CLL Mycoblasma, EBV, HIV infx immunodeficiency blood transfusion drugs
84
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?
Autoimmune hemolytic anemia. (+) Coombs
85
What laboratory test distinguishes autoimmune hemolytic anemia from Hereditary spherocytosis?
(+) Coombs indicates AIHA (-) Coombs indicates HS
86
What do you expect to find on peripheral smear of a patient with AIHA?
polychromasia, sphereocytosis, nucleated RBC
87
How do you treat Warm AIHA?
CS, rituximab, splenectomy
88
How do you treat Cold AIHA?
avoid cold, Rituxumab, plasmapheresis if refractory
89
The presence of schistocytes due to mechanical heart valve intravascular hemolysis is called what?
Fragmentation Syndrome
90
A patient who received a blood transfusion 4 hours ago is experiencing fever, allergic reactions and hemolytic sxs. What is causing this?
hemolytic transfusion reaction
91
This disease is a rare, acquired stem cell mutation characterized by complement mediated RBC lysis. What disease is this?
Paroxysmal Nocturnal Hemoglobinuria
92
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?
Paroxysmal Nocturnal Hemoglobinuria. Concern for venous thrombosis
93
You suspect your patient has PNH. What tests do you order to confirm your dx?
flow cytometry, osmotic fragility, (-) Coombs
94
How do you treat PNH?
monoclonal antibody against complement C5, steroids, stem cell transplant.