Hemepath - Anemias Part Ii (Aocd And Ncnc, Non-Hemolytic Anemias, Aplastic And Hypoplastic Anemias, And Misc)

Front 1

AOCD, iron studies typically show

Back 1

serum iron: decreased
TIBC (transferrin level): decreased (despite apparent low iron)
% tfn sat: decreased

ferritin: normal or increased!!
soluble transferrin recptor: nml

Front 2

BM in AOCD

Back 2

- erythroid precursors in normal numbers
- decreased erythroid iron
- increased storage (RE cell) iron

Front 3

AOCD is thought to be a result of __

Back 3

- prolonged physiologic adaption
- immune-driven cytokine response to deprive infectious organisms and/or tumor cells of need iron
- when prolonged, results in anemia

Effects include:
- decreased intestinal absorption of iron
- shunting of iron into macrophages, with decreased release from macrophages
- blunted EPO response

Front 4

AOCD usually develops how long after onset of chronic disease

Back 4

1-2 months

Front 5

AOCD the reticulocyte count is __

Back 5

inappropriately low in the setting of NCNC anemia with normal RDW

Front 6

Anemia with chronic renal failure

Back 6

- NCNC
- primary mech: decreased or nonfunctional EPO
- BM usually shows erythroid hypoplasia (contrast w/ AOCD)
- azotemia - direct suppression of BM and decreased RBC survival

- PB smear may show burr cells

- long-term dialysis a/w folate deficiency, RBC toxins (aluminum, copper, formaldehyde)

- pts prone to fluid overload which can further decrease HCT

Therapy: recombinant EPO and iron

Front 7

Direct toxic effects of alcohol on BM erythroid precursors

Back 7

- decreased BM cellularity
- vacuolated erythroid precursors

Front 8

Constitutional (hereditary) aplastic anemias include

Back 8

Fanconi anemia
Dyskeratosis congenita
Shwachman-Diamond syndrome

Front 9

Consitutional red cell aplasia

Back 9

Diamond-Blackfan anemia is the only well-established constitutional pure red cell aplasia

Front 10

Biochemical abn seen in Diamond-Blackfan anemia and Fanconi anemia include

Back 10

- increased HbF
- increased expression of i-antigen
- enzyme level abn

Front 11

Diamond-Blackfan tends to present at __, while constitutional aplastic anemias tend to present __

Back 11

D-B anemia: at birth or shortly after

vs.

gradual onset with progressive development of trilineage hypoplasia

Front 12

Acquired aplastic anemia (DDX)

Back 12

- idiopathic
- Secondary to drugs, toxins, infections, and other conditions
- Paroxysmal nocturnal hemoglobinuria (clonal)

Front 13

Acquired red cell aplasia (RCA)

DDX

Back 13

- transient erythroblastopenia of childhood
- parvovirus infection (usually transient)
- idiopathic pure red cell aplasia
- sustained pure red cell aplasia secondary to neoplasms, immune disorders, infections, and drugs
- antierythropoietin antibody-induced red cell aplasia in patients receiving recombinant EPO

Front 14

transient erythroblastopenia of childhood

Back 14

- self-limited red cell aplasia likely a/w antecedent viral infection (unknown)
- spontaneous recovery
- child is otherwise normal

Front 15

Main differential diagnosis when transient erythroblastopenia of childhood presents in a yound child is with __

Back 15

Diamond-Blackfan anemia

Front 16

Clinically significant parvovirus infections occur in two patient populations

Back 16

1- pts with constitutional anemias a/w decreased RBC survival times (hereditary spherocytosis, sickle cell disease)

2- pts who are immunocompromised and cannot mount an antibody response to clear the infection