Hemolytic Anemias:

Common findings in all

Decreased Hct, increased LDH, increased indirect bilirubin, increased reticulocytes, decreased haptoglobin, slightly
decreased MCV, hyperkalemia, folate deficiency
Chronic hemolysis bilirubin gallstones
Disease Presentation Associations Pathognomi Diagnosis Treatment
c findings
SCD Acute severe Bilirubin gallstones, Howell-Jones Best initial 1. Oxygen/hydrate/a
vaso-oclusive Encapsulated Bodies peripheral smear sia
crisis: acute organisms causing Most accurate- 2. Fever/leucocytosis
chest syndrome, infection, haemoglobin ceftriaxone,
pain in back and osteomyelitis electrophoresis levofloxacin or
thighs, priapism, (salmonella), LL skin Other findings- moxifloxacin
stroke, visual ulcers, avascular increased 3. Folate replacemen
disturbance necrosis reticulocytes 4. Pnemoccocal
from retinal vaccination every
infarction, +/- 5. Hydroxyurea to pr
fever recurrence of sick
Children - crises by increasin
dactylitis HgF
If severe vaso-oclusive c
give exchange transfu
Lowers mortality:
Hydroxyurea and antibio
with fever
Aplastic crisis Acute Pain crisis Sickle Cell Disease, Sudden drop Best initial test Best initial therapy for
(temporary Parovirus B-19 in reticulocyte count Parovirus B-19: IVIG
cessation of RBC Hematocrit Bone marrow giant
production) and pronormoblasts (pro-
reticulocyte erythroblasts)
count Most accurate test
for Parovirus B-19 -
PCR for DNA
Hereditary Recurrent Spherocytes Most accurate test: 1. Chronic folic acid
Spherocytosis episodes of osmotic fragility replacement to su
(abnormal round hemolysis, Other findings red cell production
shape of RBCs due intermittent abnormal round 2. Splenectomy (stop
to defect in jaundice, shape with loss of hemolysis but doe
cytoskeleton of splenomegaly, central pallor on eliminate spheroc
RBCs) Fhx of anemia or blood smear,
hemolysis, decreased MCV,
bilirubin increased MCHC,
gallstones negative Coombs
test
Autoimmune CLL, lymphoma, SLE, IgG Most accurate test: 1. Best initial therap
Hemolysis (Warm drugs: penicillin, antibodies Coombs test prednisone
or IgG) alpha-methyldopa, 2. Recurrent episode
rifampin, phenytoin; splenectomy
also associated with 3. Severe, acute
microspherocytes hemolysis not
(autoantibodies responding to
remove RBC cell prednisone IVIG
membrane leading to 4. Rituximab,
smaller membrane azathioprine,
forcing cell to become cyclophosphamide
round) cyclosporine if
splenectomy does
 work (these drugs
diminish the need
steroids in genera
Cold Agglutinin Symptoms occur EBV, Waldenstrm IgM Most accurate test: 1. Stay warm
disease in colder parts of macroglobulinemia or antibodies cold agglutinin titre 2. Rituximab and
the body such as mycoplasma Direct coombs test sometimes
numbness or pneumonia positive only for plasmapheresis
mottling of the complement 3. Cyclophosphamide
nose, ears, Smear normal or cyclosporine or ot
fingers and toes spherocytes immunosuppressiv
(Resolved by agents
warming up Steroids and splenectom
body parts) not work
G6PD deficiency African Oxidant stress due to: Heinz bodies Best initial test is for Avoid oxidant stress
(X-lined recessive American/Medite infection, dapsone, (seen on Heinz bodies and bite Irreversible hemolysis
disorder almost rranean man quinide, sulpha drugs, meythylene cells
exclusively in with sudden primaquine, blue stain) Most accurate test:
men) inability to anemia and nitrofurantoin and and bite cells G6PD level
generate jaundice with fava beans G6PD levels will be
glutathione normal sized normal after
reductate and spleen and haemolytic event;
protect RBCs from infection using must wait 1-2
oxidant stress one of the drugs months after acute
in next column episode of hemolysis
to get G6PD levels
Hemolytic Uremic Intravascular Deficiency of Schistocytes Normal PT/aPTT and Severe cases:
Syndrome (HUS) hemolysis with metalloproteinase negative Coombs plasmapheresis or plasm
and Thrombotic fragmented cells ADAMTS 13 test exchange; if there is a d
Thrombocytopenic (schistocytes), HUS associated with to phasmapheresis, infu
Syndrome thrombocytopen E.Coli 0157:H7, more FFP
Aka intravascular ia and renal freq in children
hemolysis insufficiency TTP associated with Do not transfuse platele
TTP: ticlopidine, into HUS or TTP patients
neurological clopidogrel, worsen disease)
disorders and cyclosporine, AIDS
fever (more and SLE If cases not related to dr
common in or diarrhea can use ste
adults)

Paroxysmal Episodic dark Deficiency of Decreased Best initial test: CBC 1. Best initial therap
Nocturnal urine, complement CD55 and showing prednisone
Hemoglobinuria pancytopenia regulatory proteins CD59 (decay pancytopenia and 2. Cure allogenic b
clonal stem cell and IDA, clots in CD55 and CD59 aka accelerating anemia marrow transplant
defect with unusual places Decay Accelerating factor) Most accurate test: 3. Eculizumab
increased Most common Factor; decreased CD55 and (complement inhib
sensitivity of RBCs sites of Gene for CD59 (flow for hemolysis and
to complement in thrombosis phosphatidylinositol cytometry test) thrombosis
acidosis large vessel class A (PIG-A) is 4. Folic acid and
thrombosis of defective leading to replacement with
mesenteric and over-activity of transfusions as ne
hepatic veins complement system
Most common
cause of death
thrombosis