Exam 4: Anemia Flashcards
The following lab findings are indicative of what type of anemia?
Reticulocytosis
Increased LDH
Increased indirect bilirubin
decreased haptoglobin
schistocytes on peripheral smear
Hemolytic anemia
why do you see increased reticulocytosis in hemolytic anemia?
bone marrow is trying to compensate for increased RBC destruction by releasing immature reticulocytes
What causes the increased serum LDH in hemolytic anemia?
LDH is found in RBCs, increased destruction of RBCs means increased LDH in serum
what causes the dark urine in hemolytic anemia?
increased urine hemosiderin, increased direct bilirubin
why does indirect bilirubin increase in hemolytic anemia?
increased RBC lysis overwhelms liver’s conjugation ability.
what sign may manifest as a result of increased serum indirect bilirubin?
jaundice
Why do you see decreased serum haptoglobin in intravascular hemolytic anemias?
serum haptoglobin binds to free Hb leading to low haptoglobin due to increased free Hb.
What should you expect to see on peripheral smear in hemolytic anemias?
+ schistocytes
+ reticulocytes (diff. color)
nucleated RBCs
What lab findings would indicate hemolytic anemia?
reticulocytosis
Increased LDH
Increased Indirect Bilirubin
Decreased Haptoglobin
+schistocytes and nucleated RBCs on peripheral smear
what two molecules store iron?
Ferritin and hemosiderin (macrophages)
Match the following lab values with either:
A. Iron Deficiency Anemia
B. Anemia of Chronic Disease
- Decreased serum Fe, Decreased Ferritin, Increased TIBC
- Decreased serum Fe, Increased Ferritin, Decreased TIBC
- = A
2. = B
A pt. presents c the following laboratory results.
MCV 85
Serum Fe: Decreased
Ferritin: Increased
TIBC: Decreased
What type of anemia do you suspect?
anemia of chronic disease
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
Anemia of chronic disease:
Chronically ill patients
inflammatory disease, rheum disorder, cancer, chronic infection, organ failure
how would you treat a patient presenting with anemia of chronic disease?
treat the underlying cause of anemia +/- EPO
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?
Thalassemia. As evidenced by:
-microcytic anemia (low MCV) with normal RDW, increased RBC and normal serum Fe.
What would you expect to see on peripheral smear in a pt. with one of the thalassemia
Target cells, tear drop cells, microcytosis, hypochromia
what additional test outside of peripheral smear, CBC, and iron studies can help diagnose and detect the type of thalassemia present?
Hb electrophoresis
What is the normal genotype for hemoglobin a?
aa/aa + B/B
What is the genotype in silent carrier alpha thalassemia?
aa/a- , 1 deletion
what is the genotype in alpha thalassemia minor
aa/– or a-/a- , 2 deletions
What is the genotype in alpha thalassemia intermediate?
a-/– , 3 deletions
what is the genotype in alpha thalassemia major/hydrops fetalis?
–/– , 4 deletions, fatal in utero
B-Thalassemia trait is the result of what genotype?
dysfunction in one B-globin chain
Thalassemia intermedia presents with what condition?
chronic hemolytic anemia
B-Thalassemia Major presents with what genotype?
dysfunction in both B-globin chains
What is the result of B-Thalassemia Major in hemoglobin?
excess alpha chains are unable to form tetramers leading to ineffective erythropoiesis
B-Thalassemia major presents with what kind of anemia?
severe, transfusion dependent hemolytic anemia
With hemoglobin electrophoresis, bThal presents with what?
increased hbA2 and HbF
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
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
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
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?
- Dx of IDA
2. Caused by hemorrhage, decreased Fe intake, decreased Fe absorption 2ry to celiac, h. pylori, bariatric surg.
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
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
What is the pathology of sideroblastic anemia?
Abnormal RBC iron metabolism leading to diminished heme synthesis and iron accumulation in the cells
What is the common etiology of sideroblastic anemia?
Acquired via:
chronic etoh
medications
copper deficiency
or
Congenital via:
autosomal recessive X-linked
what etiology of sideroblastic anemia is more common in adults, acquired or hereditary?
acquired
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
what are two hallmark diagnostic findings for sideroblastic anemia
ring sideroblasts on bone marrow aspirate
siderocytes with pappenheimer bodies on peripheral smear
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
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.
What differences should you expect to see on peripheral smear and bone marrow aspiration of b12 deficiency and folate deficiency?
none!
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
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
How do you treat your patient with folic acid deficiency?
1mg folic acid PO QD with food
rule out b12 deficiency
What is the most common cause of vitamin B12 deficiency/
pernicious anemia
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
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
What results from decreased intrinsic factor secretion?
decreased gastric acid secretion, and Pernicious anemia/vit. B12 deficiency
Increased risk of gastric cancer
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
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
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?
- Serum b12: decreased
- Serum methylmolonic acid: increased
- Serum homocysteine: increased
- Folate: normal
if you suspect pernicious anemia, what additional tests should be ordered?
(+) Intrinsic Factor Ab
(+) parietal cell Ab
Increased Gastrin level
(+) Schilling test
how do you treat vitamin v12 deficiency with supplementation
- Parenteral B12
Vitamin b12 1000 microgram IM/SQ QD for 1 week
Then once weekly for one month, then monthly for life
What is important to monitor when treating vitamin b12 deficiency?
potassium… new cell formation can lead to consumption of potassium
In addition to typical anemia sxs, what do you expect to see with hemolytic anemias?
Jaundice, dark urine, gallstones
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
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
why are female carriers rarely affected by G6PD deficiency?
it is an X-linked recessive disorder
what are common causes of oxidative stress that can cause G6PD crisis?
sulfa, antimalarials, aspirin
infx
fava beans
how should you treat your patient with hemolytic crisis due to G6PD deficiency?
self-limited, avoid triggers
episodic hx of hemolytic crisis
preceded by sulfa, fava, infx
Dx this…
G6PDD
sickle cells on peripheral smear
+ HbS on electrophoresis
Dx this…
sickle cell anemia
microspherocytes
Coombs NEGATIVE
(+) osmotic fragility
Dx this…
hereditary spherocytosis
(+) microspherocytes
(+) Coombs
Dx this…
autoimmune hemolytic anemia
dark urine worse in the am
Dx this…
PNH
hereditary spherocytosis is characterized by what type of inheritance pattern?
autosomal dominant intrinsic anemia
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
When treating hereditary spherocytosis with splenectomy, what should you consider?
delay splenectomy until adulthood and supplement with Folate until then
what is the hereditary pathway of sickle cell disease?
autosomal recessive
What is the genotype of sickle cell disease?
Hb SS
what is the genotype of sickle cell trait?
HbS + HbA
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
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
Aplastic crisis, pain crisis, osteonecrosis, acute chest syndrome, CVA/MI and other infarcts are common with what disease?
sickle cell anemia
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
What is the gold standard test to diagnose sickle cell disease?
Hb Electrophoresis revealing Hb S
How do you treat sickle cell disease?
avoidance of triggers
Analgesics, fluid, O2 in crisis
Transfusion if needed
Hydroxyuria
Bone marrow transplant
This disease is caused by autoAbs adhering to RBCs to induce hemolysis and complement activation.
Autoimmune hemolytic anemia
What differentiates primary and secondary autoimmune hemolytic anemia?
primary: no underlying systemic disorder
secondary: identifiable underlying systemic illness
“cold” agglutinins illicit what type of response, and are mediated by what antibody?
Acute sxs, IgM
“warm” agglutinins illicit what type of response and are mediated by what antibody?
attack at normal body temperature, IgG
What are the common causes for autoimmune hemolytic anemia?
SLE, RA
CLL
Mycoblasma, EBV, HIV infx
immunodeficiency
blood transfusion
drugs
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
What laboratory test distinguishes autoimmune hemolytic anemia from Hereditary spherocytosis?
(+) Coombs indicates AIHA
(-) Coombs indicates HS
What do you expect to find on peripheral smear of a patient with AIHA?
polychromasia, sphereocytosis, nucleated RBC
How do you treat Warm AIHA?
CS, rituximab, splenectomy
How do you treat Cold AIHA?
avoid cold, Rituxumab, plasmapheresis if refractory
The presence of schistocytes due to mechanical heart valve intravascular hemolysis is called what?
Fragmentation Syndrome
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
This disease is a rare, acquired stem cell mutation characterized by complement mediated RBC lysis. What disease is this?
Paroxysmal Nocturnal Hemoglobinuria
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
You suspect your patient has PNH. What tests do you order to confirm your dx?
flow cytometry, osmotic fragility, (-) Coombs
How do you treat PNH?
monoclonal antibody against complement C5, steroids, stem cell transplant.
