heme Flashcards
MCV values
normal - 80-100
microcytic <80
macrocytic >100
types of microcytic anemia
TICS
thalacemias
iron deficiency
chronic disease
sideroblastic
most common anemia worldwide
iron deficiency anemia
next step after dx of iron deficiency anemia
investigate cause
most common cause of iron deficiency anemia
chronic blood loss
iron deficiency anemia s/s
pica
Plummer-vinsonsyndrome (esophageal web)
atrophic glossitis
nail changes
angular stomatitis / cheilosis
blue sclera
RLS
allopecia
iron deficiency anemia labs
MCV<80
reticulocyte count low
TIBC high
serum ferritin low!!
iron deficiency anemia treatment
treat cause!!
iron supplementation
may take 6-8 weeks to correct anemia
325 mg PO TID - take with vitamin C
not all patients tolerate - N/C/D, metallic taste
can give parenteral if intolerant to AEs
anemia of chronic disease labs
MCV low or normal
- TIBC low/normal***
- ferritin high/normal***
thalassemia characteristics
autosomal recessive (need both parents)**
reduced alpha or beta chains that leads to decreased hgb synthesis, leads to hgb A displacement with abnormal types
Alpha - southeast asia/china
beta - mediterranean
thalassemia manifestations
- stunted growth, boney deformities
- splenomegaly, jaundice
- secondary hemachromatosis
- complications of iron overload
skull xray - crew cut appearance
thalassemia management
refer all to hematology and genetic counseling
SCT curative, transfuse as needed
sideroblastic anemia characteristics
congenital (X-linked) or acquired (ETOH, lead, B6 deficiency)
build up of iron in RBCs d/t inability to incorporate iron into hgb due to problem with heme synthesis
sx similar to hemochromatosis
sideroblastic anemia peripheral smear
pappenheimer bodies
ringed sideroblasts
sideroblastic anemia tx
pyroxidine, thiamine, folic acid
therapeutic phlebotomy
iron chelating agents
bone marrow or liver tx in sever cases
types of macrocytic anemias
B12 deficiency
folic acid deficiency
b12 deficiency anemia risk factors
- nutritional (vegans)
- malabsorption
- pernicious anemia (autoimmune)
- medications (PPI, H2RA, metformin)
- age >65
pernicious anemia
destruction of parietal cells that produce intrinsic factor (d/t gastric atrophy)
autoimmune
type of b12 deficiency anemia
b12 deficiency anemia manifestation not present in folic acid deficiency
complex neurologic syndrome
paresthesias, difficulty with balance/proprioception, ataxia
decreased vibratory and position sense
b12 deficiency diagnostics
- serum b12 low
- elevated serum methylmalonic acid >1000
- MCV elevated, low hct
- peripheral smear -megaloblastic
- decreased retic count
- intrinsic factor antibody, antiparietal cell antibody
b12 deficiency treatment
cyanocobalamin 1000 mcg PO daily
response in 8 weeks
if no response, can do parenteral
simultaneous folic acid replacement
referral to neurology/hematology
folic acid deficiency characteristics
macrocytic
inadequate folic acid present for DNA synthesis and RBC maturation
RF: ETOH, age>65, dietary deficient, polypharmacy, malabsorption, increased demand (pregnancy/breast feeding)
folate acid deficiency treatment
rule out B12 deficiency
ID & treat cause
folate supplement - 1-5 mg PO for1-4 mos until recovery
repeat blood work in 4-6 weeks
folate rich diet
folate acid deficiency lab findings
serum folate low - screening
RBC folic acid low - diagnostic
MCV >100
retic count normal/decreased
B12 normal
aplastic anemia
deficiency of hematopoetic stem cells resulting in pancytopenia and bone marrow aplasia with NO ABNORMAL CELLS
aplastic anemia s/s
pallor, purpura, petechiae
recurrent infections
aplastic anemia diagnostic
2 of the following:
-hgb <10
- plt <50
-ANC<1.5
smear - erythrocytes, high MCV, low/absent reticulocytes
bone marrow biopsy
aplastic anemia treatment
supportive care
severe - BMT, immunosuppression, abx/transfusions
sickle cell anemia characteristics
autosomal recessive single gene deficit in beta chain of hemoglobin A leading to chronic hemolytic anemia
hemoglobin S is sickled, leading to hypoxia & pain
sickle cell s/s
acute pain - N/V, fever, swelling, tachypnea, HTN
chronic - gallstones, jaundice, hepatomegaly, cardiomyopathy, systolic murmur, poor healing ulcers
sickle cell diagnostics
peripheral smear - sickled cells, reticulocytes, nucleated RBCs, howell-jolly bodies, target cells
elevated WbC w reactive throbocytosis
elevated indirect bili
electrophoresis - hemoglobin S
sickle cell mgmt
avoid crisis & triggers
abx prophylaxis or for chest syndrome
oxygen - sx mgmt
transfuse for vasoocclusive events
folic acid supplemetation
cure - SCT
autoimmune hemolytic anemia
acquired
caused by IgG antibody
rapid onset anemia, fatigue, dyspnea, jaundice, splenomegaly, chest pain, heart failure
autoimmune hemolytic anemia diagnostics
hgb low
retic count increased
peripheral smear - schistocytes, spherocytes, elliptocytes, spur cells, blister cells, bite cells, tear drops
indirect bili elevated
LDH elevated
haptoglobin low
direct coombs test - positive
autoimmune hemolytic anemia tx
referral to heme
removal of insult, treat underlying conditions
corticosteroids!! prednisone 1mg/kg/day, then taper
plasmapheresis
splenectomy if steroids fail
myelodysplastic syndrome
ineffective hematopoiesis: significant dysplasias resulting in one or more cytopenias
may evolve into AML
myelodysplastic syndrome risk factors
usually no cause
age, exposure to chemo, SCT, other exposures
myelodysplastic syndrome diagnostics
cytopenias
bone marrow biopsy - hypercellular marrow!!!
hereditary hemochromatosis
autosomal recessive caused by HFR (high iron) gene
hereditary hemochromatosis s/s
r/t iron accumulation!
liver disease
skin hyperpigementation
weakness/lethargy
arthropathy, arthralgias
cardiac enlargement, EKG abnormalities
impotence in males
diagnostic for hemochromatosis
liver biopsy
hemochromatosis treatment
therapeutic phlebotomy weekly
iron chelation
liver tx in severe cirrhosis
polycythemia vera
myeloproliferative disorder that causes overproduction of all three hematopoietic cell lines
RF for polycythemia vera
budd-chiari syndrome (abd pain, ascites, liver enlargement)
polycythemia vera s/s
fatigue, blurred vision, HA, dizziness
pruritis
redness o face, extremities
thrombosis - most common complication
plethora of engorged retinal veins
splenomegaly
polycythemia vera diagnostics
RBC morphology normal
high H&H, WBC, plt
JAK2 mutation confirms diagnosis
polycythemia vera mgmt
therapeutic phlebotomy
minimize risk factors
cytoreductive agent - hydroxyurea
hemophilia A & B
X- linked hereditary recessive OR acquried
reduction in coagulation factors
A - factor VIII deficiency
B-factor IX deficiency
hemophilia symptoms
muscle/joint bleeding
complications - joint destruction, transmission of blood borne infection (2/2 transfusion)
most common inherited bleeeding disorder
von willenbrand disease
von willenbrand diseae
autosomal dominant
types 1&3 (more common)- reduce quantity of circulating vWF
type 2 - reduced quality of vWF
von willebrand s/s
bleeding involving skin and mucous membranes
von willebrand labs & dx & tx
ristocetin co-factor assay
von willebrand factor antigen
prolonged bleeding time, prolonged PTT
give DDAVP, antifibrolytics, TXA, aminocaproic acid, factor VIII
ITP
autoimmune disorder causing platelet destruction
most common cause of thrombocytopenia
normal RBC, WBC
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ITP manifestations
bleeding into skin and mucosa
petechiae/purpura
ITP diagnostics
platelets <100
maybe anemia
excluse hep B, C, HIV
bone marrow biopsy
diagnosis of exclusion
ITP management
prednisone or dexamethasone
IVIG
platelet transfusion if bleeding
HIT
unfractionated heparin most likely offender
super rare in LMWH
IgG immune reaction activates platelet and promotes thrombosis in setting of thrombocytopenia
HIT RF
4 T’s
thrombocytopenia (2 pts - >50% fall, 1 pt 30-50% fall)
timing compatible w HIT (2 pts - 5-10d after heparin exposure or <1d if previously exposed, 1 pt >10 d)
thrombosis (2 - yes, 1 pt - possible)
oTher dx (2 pts - no, 1 pt- possible)
high probability: 6-8, moderate (start tx): 4-5, low 0-3
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HIT mgmt
review meds
d/c heparin
initiate DTI, then transition to Coumadin
avoid platelet transfusions
duplex doppler of lower extremities
argatroban (no liver disease)
bivalrudin (no renal disease)
fondaparinux
HIT diagnostics
4T’s probability scale
HIT antigen assay
coagulation studies
Venous doppler US
more imaging if concern for clot
DIC RF
OB
infections/sepsis (gram -)
neoplasm
massive tissue injury - trauma, burns
misc - snakebites, drugs, transfusion run, transplant rejection
DIC labs
platelet - normal, then low
fibrinogen - normal, then low
aPTT, PT - high
D dimer - high
elevated LFTs
renal dysfunction
anemia
decreased clotting factors
DIC treatment
treat underlying disorder
heme referral
transfuse goals
- platelets >20
- Fibrinogen >100 - give FFP, or cryo
- hgb >8
transfusion reaction treatment
stop transfusion
give NS
try to maintain UOP <100cc/hr
acute hemolytic rxn
usually clerical error
chills, fever, N/V,chest pain, back pain, anxiety, hemoglobinura
allergic transfusion rxn
hypersensitivity rxn to allergens in blood
pruritus, flushing, dyspnea, urticarial, angioedema
give epi, bronchodilator, antihistamines, steroids
febrile non-hemolytic transfusion reaction
multifactorial
fever without other cause for fever
give antipyretics
transfusion-related acute lung injury
granulocyte activation in pulm vasculature results in increased vascular permeability
sudden onset dyspnea, tachypnea, fever, tachycardia, hypotension
tx with supportive care, O2
delayed hemolytic transfusion reaction
fever and anemia days to weeks following transfusion
may have jaundice, hemoglobinuria
delayed hemolytic transfusion reaction
fever and anemia days to weeks following transfusion
may have jaundice, hemoglobinuria
blood transfusion reaction labs
direct antiglobulin test - Coombs test: positive in hemolytic rxn
repeat ABO testing on blood sample
UA - will show free hgb in acute hemolytic
LDH/bilirubin - may be elvated
haptoglobin - may be low
anemic hgb
women <12
men <13
iron deficiency labs
serum ferritin <40
serum iron <60
normal serum B12
> 300 normal
