Aplastic Anemia

Front 1

What's the absolute neutrophil count (ANC)?

Back 1

WBC x (%PMN + bands)

PMN < 1000: infection risk
PMN <500: high infection risk
PMN <200: opportunistic infections

Front 2

Bicytopenia vs pancytopenia

Back 2

bicytopenia: loss of two cell lines.
pancytopenia: loss of all cell lines

Front 3

What's leukopenia?

Back 3

granulocytopenia = neutropenia
lymphopenia

Front 4

What are some causes for pancytopenia?

Back 4

-aplastic anemia
-myelodysplasia (older pts, one or all cell lines)
-acute leukemia (pancytopenia with packed marrow, esp AML subtypes)
-Hairy cell leukemia (older pts, enlarged spleen, risk of opportunistic infections, treat with purine analogs)
-infiltrative process (sarcoidosis, Gaucher, osteopetrosis)
-metastatic cancer (especially breast and prostate)
-myeloproliferative disease: myelofibrosis, spent phase of PV
-drugs

Front 5

What are some causes of aplastic anemia?

Back 5

Immune-mediated (most common, attack CD34 stem cells)
drugs (chloramphenicol, carbamazpine, felbamate, phenytoin, quinine, phenylbutazone, chemo agents)
toxins (benzene, pesticides)
radiation (Hiroshima, total body irradiation, radiolabeled antibodies)
viruses (often hepatitis, EBV, HIV, CMV)
pregnancy (rare)
Congenital
Transfusion related (graft vs. host disease by lymphocytes in nonirradiated blood)

Front 6

What are the treatments available for aplastic anemia?

Back 6

immune suppression (antithymocyte globulin with cyclosporine)
stem cell transplants (HLA-matched siblings, younger individuals)
remove offending agent

Front 7

What's Fanconi anemia?

Back 7

see short stature, skeletal abnormalities, an increased incidence of tumors, sensitivity to DNA damaging agents (and aplastic anemia).
tx: stem cell transplant, androgens is BMT isn't an option

Front 8

What's PNH?

Back 8

PIGA mutation that causes hematopoeitic cells to be more susceptible to complement lysis.
20% of patients with aplastic anemia get PNH.
detected by flow cytometry (CD 55, CD59)
complications: thrombosis (Budd-chiari), eseophageal spasms, intravascular hemolysis

therapy: eculizumab (C5 antibody, decreases intravascular hemolysis)

Front 9

What are the pediatric syndromes they talked about?

Back 9

1. Thrombocytopenia with absent radius: AR, hypomegakaryocytes and thrombocytopenia, bilateral absence of radius (as in forearm)

2. Diamond-Blackfan anemia (inherited erythroblastopenia): anemia, normal WBC and platelets, hand and heart defects. tx: steroids, transfusions, BM transplant.

3. Schwachman-Diamond syndrome: AR, neutropenia, exocrine pancreatic insufficiency, short stature, increased risk of leukemia. tx: supportive and pancreatic enzymes.

Front 10

What are some causes of pure cell aplasia?

Back 10

drugs
parvovirus B19

Front 11

What's the difference between a stem cell transplant and a bone marrow transplant?

Back 11

NOTHING! two words for the same thing!!

Front 12

What are bone marrow transpants treatments for?

Back 12

aplastic anemia, thalassemias, metabolic disorders, congenital immunodeficiencies, leukemias (after meyloablative chemotherapy or chemo + radiation, with a BM transplant rescue)