Anemia

Aplastic anemia

marrow disorder characterized by reduction in the #/function of multipotential stem cells w/ pancytopenia

How would you describe the bone marrow in a pt w/ aplastic anemia?

hypocellular (patchy area of normal cellularity and inc fat cells)

Hypocellular bone marrow

normal bone marrow

What are the possible causes of aplastic anemia? (7)

- idiopathic (50%)
- chemo + radiation
- drugs (chloramphenicol)
- toxic chemicals (benzene)
- autoimmune disorders
- viral infection
- pregnancy

Most cases of of aplastic anemia present with what?

- bleeding from thrombocytopenia
- leukopenia (inc infections)
- typical anemia symptoms
- iron overload from lots of transfusions

How is aplastic anemia diagnosed?

must have 2 of the following:
- WBC < 0.5 X 10^3
- plt < 20 X 10^3
- reticulocyte < 1%

Lab findings for aplastic anemia:

- normocytic/chromic w/ normal RDW
- no normoblasts
- abs leukopenia w. relative lymphocytosis
- thrombocytopenia

What does the iron profile of a pt w/ aplastic anemia look like?

increased iron storage w/ high serum iron conc.

What levels of EPO would you expect to see in aplastic anemia?

very high EPO in plasma and urine

What are the treatment options for aplastic anemia?

transfusions, androgen/adrenal corticoids, antibiotics, marrow transplant, immunosuppressants

How does aplastic anemia affect the body long term?

PNH (paroxysmal nocturnal hemoglobinuria) in 9% of pts
MDS (myelodysplasia) and AML (acute myeloid leukemia in 16 % of pts

Fanconi's anemia

- congenital aplastic anemia
- pancytopenia detected btwn infant-8y/o

What developmental abnormalities are inherited w/ Fanconi's anemia?

- hyperpigmentation
- short (hypogonadism)
- malformation of fingers, toes, and organs
- renal disease
- intellectual disabilities

What is common in pts w/ Fanconi's anemia?

missing or extra thumb

Red cell aplasia

defect in erythroid precursors that may be related to immunological dysfunction

Acquired RCA

- arrest of erythropoiesis
- thymoma
- cytotoxic autoantibodies against eryth precursors

Congenital red cell aplasia is known as:

Diamond-Blackfan anemia

When is diamond-blackfan anemia detected?

1-6 years old

What cells are affected in congenital red cell aplasia?

BFU-E and or CFU-E decreased

Lab findings for diamond-blackfan anemia

- severe normo anemia
- low retic count
- normal WBC and plt
- marrow reduction in all erythroid cell production

Anemia of Chronic Disorders

RBC production is normal but insufficient to compensate for dec survival of RBCs

In what disease states does anemia of chronic disorders occur?

- infection (50%) TB, lung abcess, bact endocarditis
- malignancy
- RA
- SLE

What is the main cause of anemia of chronic disorders?

hepicidin induced by inflammation

What does hepcidin do?

controls iron absorption in small intestine and stored iron recycling in macrophages

Lab findings for anemia of chronic disorders

- normocytic/chromic
- anisocytosis
- dec serum Fe

Anemia of renal insufficiency

- end stage renal disease
- dec EPO production
- dec RBC survival
- dec plt function

How would you assess the severity of anemia of renal insufficiency?

elevation of BUN

Anemia of liver disease

- target cells, acanthocytes, macrocytosis
- inc retic
- LFTs correlate w/ liver disease

Lab findings for anemia of liver disease

INC ALT, AST, GGT, MCV

Anemia of endocrine disease

occurs w/ hypothyroidism and androgen deficient states

Hypothyroidism associated anemia lab findings

- low retic
- dec thyroid hormone = lower tissue O2 need = dec EPO
- macro or normocytic and normochromic

Testosterone deficiency associated anemia

dec RBC production and drop in Hgb

Myelophthisic anemia

bone marrow infiltration and hyperproliferation by non-erythroid cells (normocytic/chromic)

Causes of myelophthisic anemia

metastatic carcinoma, MM, leukemia, lymphoma, lipidoses, or storage disease

Myelophthisic anemia is commonly accompanied by what?

leukoerythroblastic rxn (immature erythroid and myeloid cells)