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*Pance Re-Up * > Hematology #2 (Normocytic Anemias) > Flashcards

Hematology #2 (Normocytic Anemias) Flashcards

1
Q

MC type of normocytic anemia

A

Anemia of chronic disease: decreased RBC production in setting of chronic disease

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2
Q

There are three main factors that decrease serum iron in anemia of chronic disease. What are they?

A

1) Increased hepcidin: blocks release of iron from macrophages and reduces GI absorption of iron
2) Increased ferritin: sequesters iron into storage
3) Erythropoeitin inhibition via cytokines (Erythropoeitin stimulates RBC synthesis)

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3
Q

What does a CBC for anemia of chronic disease show?

How about iron studies?

A

Normocytic normochromic anemia

Decreased serum iron + increased ferritin + decreased TIBC

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4
Q

Treatment for anemia of chronic disease

How about if renal disease?

A

-Treat underlying disease to correct anemia

-Erythropoeitin-alpha

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5
Q

Explain how to differentiate the iron studies in the following anemias:

Iron deficiency
Anemia of Chronic Disease
Thalassemia

A

-Iron deficiency: decreased ferritin, increased TIBC
-Chronic Disease: increased ferritin, decreased TIBC
-Thalassemia: normal or increased serum iron

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6
Q

What is G6PD deficiency? What are some risk factors for it?

A

X-linked recessive disorder of RBC’s that causes episodic hemolytic anemia

Males, African Americans

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7
Q

What is the pathophysiology of G6PD deficiency and how does this affect RBC’s?

A

Normally, G6PD generates NADPH to protect the RBC’s from oxidative stress. In deficiency, methemoglobin is produced which leads to Heinz Bodies (clumps of damaged hemoglobin attached to RBC’s)

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8
Q

What are some exacerbating factors for G6PD deficiency?

A

-Infection (DKA) = MCC
-Fava Beans
-Meds (Dapsone, Nitrofurantoin, Chloroquine, Sulfa Drugs)

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9
Q

What are the symptoms of G6PD deficiency and what is unique about them?

A

-Asymptomatic until times of oxidative stress
-Episodic hemolytic anemia 2-4 days later
–back or abdominal pain, anemia
–jaundice (dark urine)

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10
Q

What is seen on peripheral smear and CBC in a patient with G6PD deficiency?

A

-Heinz bodies, schistocytes (bite cells)
-Normocytic hemolytic anemia during crisis

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11
Q

What other lab can you do for G6PD deficiency?

A

Enzyme assay for G6PD after episodes

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12
Q

Treatment for G6PD deficiency

A

-Avoid offending foods and drugs
-Iron and folic acid supplements
-Phototherapy if severe jaundice/neonatal

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13
Q

Sickle Cell Disease affects what part of the RBC?

Explain the pathophysiology of this.

A

-Beta-globin chain (RBCs sickle, cause hemolysis, and a vaso-occlusive crisis)

Point mutation where valine substitutes for glutamic acid in the beta chain. Sickle hemoglobin (HbS) has decreased solubility under hypoxic conditions, leading to sickling, vaso-occlusion, and hypoxia. Sickle cells are destroyed by the spleen (hemolytic anemia)

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14
Q

What is Sickle Cell Trait (Heterozygous AS)?

A

8% of African Americans

-Ususally asymptomatic unless exposed to extreme physical stress, extreme hypoxia, high altitudes, or dehydration.

-May develop episodic hematuria or isosthenuria (CKD, neither concentrated or dilute urine)

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15
Q

What is seen on hemoglobin electrophoresis in a patient with Sickle Cell Trait?

A

-Presence of both hemoglobin A (HbA) and Hemoglobin S (HbS) with A > S

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16
Q

Treatment for Sickle Cell Trait

A

Normally doesn’t need treatment

17
Q

On the other hand, Sickle Cell Disease is….

A

-Homozygous sickle mutation (SS)

18
Q

When do symptoms begin in patients with Sickle Cell Disease, why, and what is the MC initial presentation?

A

As early as 6 months (when HbSS replaces fetal hemoglobin)

Dactylitis MC initial presentation
-Others: Delayed growth, fever, infections

19
Q

Explain increased risk of infections in patients with Sickle Cell.

A

-Functional asplenia and autosplenectomy (from repeated splenic infarctions) leads to increased risk of infections with encapsulated organisms (S. Pneumo, H. Flu, GABHS, etc.)

20
Q

What organism should you be concerned about in a patient with Sickle Cell who gets Osteomyelitis?

A

Salmonella spp.

21
Q

Asplastic crisis (bone marrow stops making RBC’s) is associated with what infection in those with Sickle Cell Disease

A

parvovirus B19 infections

22
Q

What are some symptoms of a vaso-occlusive crisis in those with Sickle Cell Disease, or other symptoms they may be having?

A

-Acute splenomegaly and rapid decrease in Hgb (RBC pooling in spleen)
-Jaundice, Pigmented gallstones
-Acute chest syndrome, back, abdominal, bone pain
-Priapism **
-Avascular (ischemic) necrosis of bones (femoral or humeral head). H-shaped vertebrae
-Tibial skin ulcers
-Stroke**

23
Q

Best initial test for Sickle Cell Disease. What is seen on it?

A

-Peripheral Smear: Target cells, sickled erythrocytes, decreased hemoglobin, decreased hematocrit

Howell-Jolly bodies (nuclear remnants) that indicate functional asplenia

24
Q

What is seen on hemoglobin electrophoresis in those with Sickle Cell Disease?

A

-HbS, little to no HbA, increased HbF (it has better affinity for oxygen than HbA)

25
Q

In Sickle Cell Trait, what is seen on electrophoresis?

A

HbS, decreased HbA

26
Q

What is the definitive diagnostic for Sickle Cell Disease?

A

DNA analysis

27
Q

What is the treatment for a patient in a Sickle Cell Crisis?

A

-Pain Control: IVF and oxygen (reverses and prevents sickling)
-Folic acid supplement (to promote RBC production)
-RBC transfusion may be needed

28
Q

What is the only potentially curative treatment for Sickle Cell Disease, even though it has significant side effects?

A

Allogeneic stem cell transplant

29
Q

In order to reduce episodes of Sickle Cell crisis, what medication can be used and what does it do?

A

Hydroxyurea: increases production of HbF (which does not sickle and has a higher affinity for oxygen) and reduces RBC sickling

30
Q

Is Hydroxyurea used for acute episodes?

A

No, because it takes weeks to months to take effect

31
Q

Because patients with Sickle Cell Disease are prone to more infections, what is given prophylactically to prevent complications?

A

Penicillin as early as 2-3 months until 5 years old to prevent complications

32
Q

What vaccines should someone with Sickle Cell Disease FOR SURE get?

A

Pneumonia and Influenza Vaccinations

33
Q

What does the word “Pancytopenia” mean?

A

Low WBC’s
Low Platelets
Low RBC’s
Low Hgb

34
Q

What is aplastic anemia? (think about the name)

A

-Bone marrow hypocellularity (reduced number of hematopoietic cells) with pancytopenia (low number of RBC’s, platelets and WBC’s)

35
Q

Etiologies of aplastic anemia

A

-Idiopathic MCC
-Radiation
-Infections: Parvovirus B19
-Meds: Sulfa drugs, Chloramphenicol, Anti-Epileptics (Carbamazepine, Phenytoin), NSAIDs, Anti-Thyroid Meds

36
Q

What are some symptoms of pancytopenia

A

-Easy bruising, bleeding, frequent infections, fatigue
–Thrombocytopenia (low platelet count): mucocutaneous bleeding
–Anemia: weakness, fatigue, dyspnea
–Fever, Recurrent infections

37
Q

What is seen on CBC with peripheral smear in aplastic anemia?

A

-At least 2 cytopenias: few or absent reticulocytes, thrombocytopenia (low platelets), neutropenia (few WBC’s), anemia (few RBC’s)

38
Q

What’s the most accurate test for aplastic anemia? (Think about what is affected)

A

Bone marrow biopsy: hypo cellular, fatty bone marrow (T cells attack hematopoietic stem cells and bone marrow is replaced with fat)

39
Q

Treatment for aplastic anemia

A

-Supportive (initial): Broad spectrum ABX, PRBC transfusion

-Severe in otherwise healthy patients < 50 y/o: Allogeneic hematopoietic stem cell transplant***

-Immunosuppresive therapy if > 50 years old or if not matched donor: Eltrombopag, Cyclosporine, Prednisone

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