Heme JB Flashcards
alpha thalassemia labs
CBC: hypo chromic microcytic anemia. normal/incr RBC, normal/inc serum iron and iron stores. hgb low
BPS: target cells, Heinz bodies
Hb electrophoresis
alpha thalassemia tx
mild (alpha): no tx
mod: folate if reticent high, avoid oxidative stress, avoid iron
severe: blood transfusion weekly, Vit C, folate. iron chelating agents. splenectomy.
bone marrow transplant definitive
anemia of chronic disease labs
decreased serum iron
increased ferritin
decreased TIBC
intrinsic (inherited disorders) in hematology
sickle cell
thalassemia
G6PD
hereditary spherocytosis
extrinsic (acquired disorders) in hematology
autoimmune hemolytic anemia
DIC
TTP
HUS
paroxysmal nocturnal hemoglobinuria
hypersplenism
autoimmune hemolytic anemia tx
warm (IgG ab): CS 1st line –> splenectomy or rituximab –> immunosuppressants, IVIG
cold (IgM ab): avoid cold, rituximab. plasmapheresis if refractory
Coombs + distinguishes it from hereditary spherocytosis
beta thalassemia sx
minor: asx
intermedia: assoc w/ anemia, hepatosplenomegaly, and bony disease
major (Cooleys anemia)L asx @ birth but sx @ 6mo. frontal bossing, maxillary overgrowth, hepatosplenomegaly, severe hemolytic anemia, osteopenia, iron overload, pigmented gallstones
beta thalassemia dx
CBC: hypo chromic microcytic anemia, normal/inc RBC and serum iron.
PBS: target cells
electrophoresis: low HbA, high HbA2, high HbF
xray: skull bossing w/ hair on end appearance
beta thalassemia tx
minor: no tx, genetic counseling
major/severe: periodic transfusions, VitC and folate supplementation
avoid excess iron intake. iron chelating agents. splenectomy if refractory.
allogenic bone marrow transplant definitive
CML sx
plastic crisis: acute leukemia (splenomegaly, + Philadelphia t9:22, increased WBC)
Protein C deficiency sx
recurrent DVT and PE
Protein C deficiency tx
heparin –> oral coagulation for life
DIC sx
bleeding out and clotting
widespread hemorrhage at venipuncture sites, mouth, nose, extensive bruising
thrombosis
DIC tx
tx underlying
severe bleeding –> FFP + cryoprecipitate +/- plt transfusion
severe thrombosis –> heparin
anticoagulants drug mechanism
heparin: intrinsic pathway; PTT/ factors 8, 9, 11, 12
warfarin: extrinsic pathway; PT/INR, factors 2, 7, 9. 10
factor V leiden sx
hx of pregnancy loss, DVTs or unprovoked DVTs
factor V leiden dx
activated protein C resistance assay
if positive, confirm w/ DNA testing
normal PT/PTT
factor V leiden tx
high risk: indefinite anticoagulants. may need thromboprophylaxis during pregnancy to prevent miscarriage
moderate risk: (thrombotic event w/ prothrombotic stimulus or asx) prophylaxis during high risk procedures
G6PD sx
African American
acute hemolysis from infection, sulfa drugs, or fava beans
jaundice, pallor, dark urine
G6PD dx
PBS: schistocytes (bite cells); Heinz bodies
G6PD tx
self limiting
good pasture syndrome sx
IgG abs against type IV reaction
glomerulonephritis (rapidly progressing) +
pulmonary hemorrhage (hemoptysis)
good pasture syndrome dx
bx: linear IgG deposits in glomeruli or alveoli
+ anti-GBM ab
good pasture syndrome tx
CS + cyclophosphamide
plasmapheresis
hemochromatosis sx
arthritis
bronzing of skin
cirrhosis
hemochromatosis dx
increased serum iron, increased serum transferrin saturation. normal/dec TIBC, increased ferritin
+/- increased LFT
liver bx gold standard: increased liver parenchymal hemosiderin
hemochromatosis tx
phlebotomy weekly until ferritin dec, transferrin dec, or mild anemia
maintenance phlebotomy 3-4x/yr for life
chelating agents if unable to do phlebotomy
hemolytic anemia tx
glucocorticoid and cytotoxic drugs
hemolytic anemia secondary to renal failure tx
hemodialysis
hemolytic anemia secondary to renal failure sx
red/brown urine and plasma
low haptoglobin, high LDH
HUS sx
kid with GI sx (e. coli, shigella, salmonella)
anemia, kidney damaged (inc BUN/Cr)
thrombocytopenia (petechiae, bruising)
no neuro sx or fever like in TTP
.
HUS tx
observation, usually self limited, IV fluids
plasmapheresis if severe, neuro complications, non-renal complications
abx may worsen sx
hemophilia A sx
factor 8 deficiency
intrinsic pathway
hemarthrosis, excessive hemorrhage in response to trauma and surgery/incision
hemophilia A dx
low factor 8
prolonged PTT
mixing study w/ normal plasma will correct PTT
hemophilia A tx
factor 8 infusion
DDAVP before procedures
hemophilia B sx
Christmas disease
deficiency in factor 9
deep tissue bleeding
hemophilia B dx
decreased serum factor 9
prolonged PTT
hemophilia B tx
factor 9 infusion
DDAVP not useful
HSP sx
MC children after URI
HA, F, anorexia –> rash (legs, symmetrical, palpable purpura)
abd pain, subcutaneous edema, hematuria, proteinuria
HSP dx
mainly clinical
kidney bx: mesangial IgA deposits
normal coags (PT/PTT)
normal plt
HSP tx
self limiting
NSAID for joint pain
hereditary spherocytosis sx
anemia, jaundice, hepatosplenomegaly
pigmented black gallstones (calcium bilirubinate)
hereditary spherocytosis dx
PBS: hyper chromic microcytosis (RBC lacking central pallor)
+ osmotic fragility test
- Coombs test
increased MCHC
hereditary spherocytosis tx
folic acid
splenectomy TOC in severe dz
hodgkin dz sx
20yo or 50yo
painless lymphadenopathy in upper body, hepatosplenomegaly
pel ebstein fever: cyclical fever that increases and decreases over 1-2wks
night sweats, wt loss, anorexia
reed Sternberg cells
associated with EBV (mono)
ITP sx
usually asx
increased mucocutaneous bleeding: purpura, bruises, petechiae, epistaxis
no splenomegaly
acute: MC in children after viral infx (self limiting)
chronic: MC in adults (often recurrent)
ITP dx
isolated thrombocytopenia w/ normal coags
PBS: may show megakaryocytes
ITP tx
child: observation +/- IVIG
adults: CS +/- IVIG, splenectomy if refractory
plt transfusion if <20,000
iron deficiency anemia labs
decreased serum iron, decreased ferritin
increased TIBC, decreased RDW, decreased RBC/Hgb/Hct
decreased MCV, decreased transferrin saturation
decreased reticulocytes
iron deficiency anemia tx
ferrous sulfate PO daily
type of bilirubin that is always pathalogic
increased direct bilirubin
CLL sx
adults, asx
fatigue, DOE, increased infx, painful lymphadenopathy
hepatosplenomegaly
smudge cells
multiple myeloma sx
bone pain
recurrent infx
hypercalcemia, anemia, kidney failure
multiple myeloma dx
M protein spine on electrophoresis
Bence jones proteinuria
Rouleaux formation –> increased ESR
punched out lytic lesions on skull xray
bone marrow bx: plasmacytosis
multiple myeloma tx
stem cell transplant definitive +/- chemo or alkylating agents (melphan)
bony destruction: bisphosphonates (alendronate)
paroxysmal nocturnal hemoglobinuria tx
eculizumab (anti complement CD5 ab)
prednisone decreases hemolysis
marrow transplant
pernicious anemia dx
increased MCV
decreased B12
+ intrinsic factor Ab, parietal cell ab
increased gastrin levels
+ schilling test
pernicious anemia watch for which lab during tx
signs of hypokalemia
polycythemia vera dx
screening: Hgb >16, Hct >48%
diagnostic test: subnormal EPO and JAK2 V617F or exon 12 mutation
polycythemia vera (primary erythrocytosis) caused by
JAK2 mutation
polycythemia vera (primary erythrocytosis) sx
increased Hct without hypoxia
HA, dizzy, tinnitus, blurry vision
pruritus after hot bath
episodic burning/throbbing of hands and feet w/ edema
splenomegaly, facial plethora
polycythemia vera (primary erythrocytosis) tx
therapeutic: phlebotomy (management of choice) until Hct <45%
low dose aspirin to prevent thrombosis
myelosupression: hydroxyurea, IF alpha
allopurinol if hyperuricemic
ruxolitinib
secondary polycythemia sx
increased hct d/t another process
cyanosis, clubbing, HTN, hepatosplenomegaly +/- heart murmur
hx of COPD, renal cell carcinoma, dehydration
secondary polycythemia dx
increased RBC/hct w/ normal WBC and plt
normal WBC/plt is difference between primary and secondary
secondary polycythemia tx
tx underlying, quit smoking
sickle cell dz aplastic crisis caused by
parvovirus B19
Sickle cell dz dx
CBC w/ PBS: dec Hgb, dec hct, increased reticulocytes
Howell jolly bodies
sickle cell dz tx
pain management- IV fluids, O2, folic acid, +/- RBC transfusion
vaccinations for sickle cell dz
children: S. pneumococcus, Hib, N. meningococcus
prophylaxis penicillin 4mo-6yo
TTP sx
pentad:
thrombocytopenia
microangiopathic hemolytic anemia
kidney failure
neuro sx
fever
increased incidence w/ HIV
