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80 Cards in this Set

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What is hemolytic anemia definition
Short red blood cell (RBC) life span
- associated with accumulation of hemoglobin catabolism due to RBC destruction
- associated with inc erythropoiesis and reticulocytosis
RBCs made where
bone marrow
RBCs survive how long
100-120 days
Early RBCs have ribosomes, stain with reticulin called
normal reticulocyte %
with early hemolysis we see what reticulocyte %
higher % >1%
Consequences of RBC hemolysis
Anemia – pallor, shortness of breath, etc
Expansion of marrow space w/ skeletal deformity
**(tower skull)
Growth retardation
Funny looking RBC
Increased reticulocytes
Increased bilirubin, uric acid, and LDH
Bilirubin gall stones
First step to diagnosis of hemolytic anemia
First step: identify presence of Hemolysis
Hemolytic anemias have what shape and size RBCs?
- sickle
- spherocyte
- shistocyte
- acanthocyte
- stomatocyte
- Heinz bodies
– liver disease, abetaliproteinemia, postsplenectomy
Basophilic stippling
– lead tox, thalassemia
Bite cells
– G6PD def, oxidant drug hemolysis
Burr cells
– uremia, ulcers, gastric CA
Cabot’s ring
– splenectomy, megaloblastic, hemolytic
Howell-Jolly bodies
- splenectomy, megaloblastic, hemolytic
Lab Diagnosis of hemolytic anemia look for
Low HB < 12 g/dl
Low Hct < 33%
reduced number of RBCs
Blood smear
---normochromic normocytic
---microcytic hypochromic (MCV < 80 fL)
---macrocytic (MCV>100 fL)
RBC shape, fragments….
Reticulocyte count
Key diagnostic features of Hemolytic Anemia?
Blood smear: hypochromic microcytic anemia
Signs of heme degradation
What is Extravascular Hemolysis
removal of RBC by the Reticuloendothelial system (spleen macrophages)
What is intravascular hemolysis?
destruction of RBC in the circulation with free hemoglobin release.
Lab values for intravascular hemolysis
Inc Indirect and direct Bilirubin
Inc Free hemoglobin
Haptoglobin depleted
Inc Methemoglobin
--Bilirubin (common)
Intravascular hemolysis is associate with what immune activation
complement activation
- thus we see the free hemoglobin release
- Buffer proteins that transport hemoglobin (haptoglobin) is thus dec
Lab values for extravascular hemolytic anemia
--Direct Bilirubin
--Haptoglobin low
--Bilirubin RARE
Second step in making diagnosis of hemolytic anemia?
Identify the mechanism of hemolysis
---Intrinsic defect or intracorpuscular
---Extrinsic defect or extracorpuscular
Intrinsic hemolytic anemias include
1- Abnormal RBC membrane
***spherocytosis, elliptocytosis
2- Enzyme disorder
***G6PD or pyruvate kinase
3- Abnormal Hemoglobin
***Sickle cell, Thalassemias
External factors include
antibodies and thrombosis
Key membrane abnormalities
Hereditary spherocytosis
Hereditary elliptocytosis
Paroxysmal nocturnal hemoglobinuria
Hereditary spherocytosis is what
Defect of the RBC membrane (mut in ankyrin protein)
- renders the erythrocytes spheroidal
- susceptible to splenic sequestration and destruction
- autosomal dominant
Spherocytosis is a ____vascular mechanism
extravascular mechanism of HA
hereditary spherocytosis
defects in membrane to cytoskeleton binding lead to membrane separation and loss as microvesicles. Small dense microspherocytic RBCs result that have reduced osmotic fragility.
Diagnosis of spherocytosis
1) Blood smear:
Anemia, Spherocytosis
2) Osmotic fragility test 
3) Bone marrow:
increased erythropoiesis,
4) Clinically:
jaundice (Bilirubin), splenomegaly
Management of Spherocytosis
Because of the hyperactive bone marrow
To prevent aplastic anemia, Folic Acid treatment is recommended
Splenectomy in symptomatic patients
Hereditary Elliptocytosis
Autosomal Dominant inherited defect
Elliptical RBC and variation in RBC shape (Poikilocytosis)
Paroxysmal nocturnal hemoglobinuria (PNH)
-- hyperactivation of the complement system
RBC membrane defect of :
--- Decay accelerating factor (DAF),
--- CD59 (membrane inhibitor of lysis)
both factors protect RBC from complement damage
Patients with PNH have heightened sensitivity of complement because of the loss of ____
What test shows inc sensitivity to complement
sucrose hemolysis test
Inherited enzyme deficiencies for HA
Glucose-6-Phosphate Dehydrogenase (G-6-PD) Deficiency (X-linked)
What happens in G6PD deficiency
RBC membranes are sensitive to oxidants;
inability to neutralize oxidation.
How to diagnose enzyme defects
G-6-Pd is assessed by submitting RBC to oxidative stress
- may be a result of antimalarial drugs, primaquine
----trigger HA after 3 days of therapy
- Other : sulfonamides, nitrofurantoin, fava beans
Heinz bodies are seen in
- denaturation with precipitates
- also see bite cell formation
Pyruvate kinase deficiency
(autosomal recessive)
Involving the energy-producing step of the glycolytic pathway, defective
result in extravascular hemolysis in the spleen.
RBC microvesiculation is related to
low ATP concentrations is seen with PK deficiency
Diagnosis of HA wrap up: Steps 1 and 2
First step: identify presence of Hemolysis
Second step: identify the mechanism of hemolysis
intrinsic defect or intracorpuscular
extrinsic defect or extracorpuscular
Extrinsic Hemolytic Anemia are typically
Non immune mediated
Immune Mediate
Non immune hemolytic anemia typically classified as
mechanical hemolysis
Non immune hemolytic anemia seen with
Physical trauma
- Prosthetic heart valves (macroangiopathies)
- HUS, TTP, DIC (microangiopathies)
Schistocytes, fragmented RBCs
Microangiopathic are associated with
macroangiopathic are associated with
prosthetic valves
Mechanical valves cause hemolysis how?
- RBCs hitting the metal
--also see infusion pump generated hemolysis
Diagnosis of TTP- thrombotic, thrombocytopenic, purpura
F - Fever
A - Anemia
T - Thrombocytopenia
R - Renal dysfunction--elevated creatinine
N - Neurologic changes
TTP - Thrombotic Thrombocytopenic Purpura what is the modern DX
Elevated LDH
elevation of vWF in circulation
what is generally considered the cause of the hemolysis in TTP
Low platelet count is considered the cause of the hemolysis
Treat TTP with
Plasma exchange
Non- immune/non-mechanical causes of HA
Infection (Malaria, Babesia, Streptococcus, H. I., Clostridium Perfringens)
Chemical trauma (medications)
Hypersplenism (increased phagocytosis)
Hemolytic Anemia: Infections
Babesiosis---martha's vineyard
Bartonellosis (Oroya fever)
Clostridium Perfringens
***look for travel history
Hemolytic Anemia: Chemical Exposures
Strong oxidants - UDMH
Oxidants with G-6-PD deficiency
Snake venom
Chlorine in hemodialysis fluid
Chloramine in hemodialysis fluid
**stop the exposure
Hemolytic Anemia: key associated organ diseases
Liver Disease
Extrinsic Hemolytic Anemia can be
Non immune mediated
Immune Mediated
Transfusion related hemolysis is an
immune hemolysis
Examples of Immune mediated hemolysis
Transfusion-based immunity
-- ABO incompatibility
-- Delayed transfusion reactions caused by Rh, Kell, Duffy, Kidd, etc
Hemolytic disease of the newborn
Autoimmune hemolytic anemias
Ultimate test to understand the HA is the
Coombs Test
- will identify an immune antibody attack of RBC surface
Symptoms of immune mediated hemolysis
fever, hypotension, hemoglobinuria, flank pain, constriction of chest
Acute hemolytic transfusion reactions, frequency and symptoms:
1 in 600 to 1/ 25K components
death associated with ABO mismatch
Clinical Findings: pain at site of infusion, chest pain, flank pain, back pain, hypotension,hemoglobinemia, hemoglobinuria, hypotension, acute renal fail, DIC
Only need a few ccs of incompatible cells to cause
shock, renal failure, death...
Acute hemolysis is due to
due to complement binding and activation
in immune mediated hemolysis testing hemosideriuria is obsolete
but drops and then rises in a few days
in immune mediated hemolysis hemoglobin will
rise and fall over a couple days
Acute vs delayed hemolytic transfusion reactions
Acute: Usually ABO antigen mismatch, IgM antibodies, and intravascular hemolysis +/- complement anaphylatoxins leading to shock
Delayed: Minor antigens, IgG antibodies, and extravascular hemolysis.
Red cells coated with antibodies are hemolyzed by what cells and where
- macrophages in the spleen
Alloimmune hemolytic anemia.
(due to allo antibodies)
induced by transfusion, pregnancy
Autoimmune hemolytic anemia
(due to auto antibodies)
inappropriate immune response
2 types of HA due to autoantibodies
- warm reacting at 37 deg
- cold reacting only active at 30 deg or less
Warm AIHA at 37 C
extravascular hemolysis, IgG mediated, through phagocytosis in the spleen
IgG circulating in autoimmune disease (SLE)
Drug induced IHA
methyl-dopa, penicillin, quinidine and salicylates
Cold AIHA below 30 C
intravascular, IgM mediated, complement mediated
associated with infections
(mycoplasma pneumoniae, antibody directed against I-antigen)
Cold agglutinin disease and Raynaud ‘s Phenomenon
Acquired membrane defect causing AIHA
Paroxysmal Cold Hemoglobinuria
Paroxysmal Cold Hemoglobinuria see
IgG antibody directed against P- RBC antigen, binds at cold temperature,
binding complement, intra vascular hemolysis, active at 37 C
Hemolytic Disease of the Newborn
*Mother exposed to fetal RBC antigens which she lacks and to which she forms a humoral immune response (other than ABO Ag)
*Maternal IgG antibodies cross the placenta and hemolyze fetal RBCs
Elevated bilirubin
Fetal anemia
Fetal compensatory extramedullary hematopoiesis
Mother’s Blood type is: A Rh (+)
Baby’s Blood type is: O Rh (+)
Baby Red blood cells are compatible with Mom?
Baby’s RBCs are coombs positive
Baby is Jaundiced and anemic (Hct 25%)
Hydrops Fetalis & Hemolytic Disease of the Newborn
Antigen neg mom
Sensitized to antigen pos baby by fetomaternal hemorrhage or previous pregnancy
Makes antibodies
cross the placenta
Hydrops Fetalis & Hemolytic Disease of the Newborn Treatments
Intra-uterine and/or Exchange Transfusion
- Select Rare RBCs ABO Compatible but negative for Antigen which is present on baby’RBCs
Hemolytic anemias definition
“Decreased levels of erythrocytes circulating in the blood (anemia) because of their increased destruction (hemolysis)”