Hematology

Question 1

Leukemia
Path:
Pt:
Dx:
Tx:

Answer 1

AML
Path: acute= blasts, myelogenous= neutrophils
Pt: acute, age 67
Exposure: benzene radiation
Dx:
smear: blasts
BM bx: >20% blasts
\+myeloperoxidase
Tx:
M3: vitamin A (Auer Rods on bx)
Not M3: chemo 

ALL
Path: Acute= blasts, Lymphocytic= lymphocytes 
Pt: acute, age 7
Dx:
smear: blasts
BM bx: >20% blasts
\+cALLa and +TdT
Tx:
Chemo
PPx CNS, +/- radiation

CML
Path: Chronic=mature cells, myelogenous= neutrophils
Pt: chronic, age 47
Dx:
Diff (way more cells then should be)
BM bx: philadelphia +t(9,22), +BCR-ABL
Tx:  Imatinib (tyrosine kinase)

CLL
Path: chronic=mature cells, lymphocytic=lymphocytes 
Pt: chronic, age 87
Dx:
Diff (way more cells then should be)
BM bx
Tx:
>65 + asx = nothing
>65 + sx= chemo
<65 + donor= stem cell transplant

Question 2

Anemia of chronic dz
Path:
Dx:
Tx:

Answer 2

Path: chronic inflammatory dz

Dx: Microcytic anemia
Iron: low
TIBC: low
Ferritin: high

Tx: EPO, tx inflammatory dz

Question 3

Microcytic anemia path
dx
tx

Answer 3

iron deficiency anemia
Iron: low
TIBC: high
Ferritin: low
tx: po iron

anemia of chronic disease
Iron: low
TIBC: low
Ferritin: high
Tx: chronic dz control, EPO

thalassemia 
Iron: normal
Ferritin: normal
TIBC: normal
Hbg electrophoresis 
Tx: Major: transfusion, deferoxamine

sideroblastic
Iron: high
TIBC: normal
Ferritin: normal
best: bone marrow bx-> ringed sideroblasts
tx: remove exposure (copper, lead, ETOH), give back B6, treat cancer

Question 4

Macrocytic anemia path
pt
dx
tx

Answer 4

folate deficiency 
pt: tea + toast diet, pregnancy, ETOH
dx:
low folic acid
normal methylmalonic acid 
tx: folate

B12 deficiency
pt: vegan, crohn's dz, s/p gastric bypass
Dx:
low B12 levels
high methylmalonic acid
tx: B12

Pernicious anemia

pt: ab against parietal cells, no intrinsic factor, no b12 absorption
dx: ab-intrinsic factor or anti-parietal
tx: IM b12

Non-megaloblastic anemia
Pt: liver dz, ETOH, meds-> AZT, HAART, 5-FU

Question 5

Normocytic anemia
path:
dx:
tx:

Answer 5

Sickle Cell
dx: smear-> sickled cells
hgb electrophoresis-> SC,SS
tx: hydroxyurea, IVF, O2, Pain control, exchange transfusion (emergency)

G6PD- african american males, dapsone, TMP-SMX, nitrofurantoin

dx: smear-> bite cells, heinz bodies
best: G6PD levels 6-8 weeks
tx: supportive, avoid stress

Hereditary spherocytosis
dx: smear-> spherocytes
best-> osmotic fragility
tx: folate + fe, splenectomy

Autoimmune hemolytic anemia 
Warm:
dx: + coombs test IgG
tx:  steroids, splenectomy, severe-> IVIg
Cold:
dx: negative coombs 
tx: avoid cold, refractory: rituximab

Question 6

Clotting disorders
path
pt
dx:

Answer 6

Von Willebrand disease: MC inherited bleeding disorder; dec VWF, dec factor III
Pt: Easy bruising, skin bleeding, prolonged bleeding from mucosal surfaces-> gingival, vagina, skin
Labs:
Normal Plt
Normal PT/PTT
Normal aPTT
Dx: VWF assay
Tx: Desmopressin (DDAVP); give factor if severe hemorrhage

Hemophilia A
Pt: hemarthrosis, hematoma 
Labs:
Normal Plt
Normal PT/PTT
Elevated aPTT
Decreased factor VIII level 

Hemophilia B
Pt: hemarthrosis, hematoma
Labs:
Normal Plt
Normal PT/PTT
Elevated aPTT
Normal factor VIII level 
Decreased factor XI level

Question 7

G6PD Deficiency Anemia 
Path:
Pt:
Dx:
Tx:

Answer 7

Path: Low G6PD
X-linked recessive pattern of inheritance
Hypoxemia = hemolysis

Pt:
African American males
dapson, TMP-SMX, nitrofurantoin

Dx: Normocytic anemia
Smear = bite cells, Heinz bodies
Best-> G6PD level 6-8 weeks after exposure

Tx:Supportive
Avoidance of oxidative drugs