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43 Cards in this Set

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What are the 2 classifications of Hemolytic Anemias

Intracorpuscular defects




Extracorpuscular defects

RBC membrane defects




Enzyme deficiencies




Hemoglobinopathies




Thalassemias




Is the Intracorpuscular or Extracorpuscular




Is it inherited or Acquired

Intracorpuscular defect




Inherited

Paroxysmal nocturnal hemoglobinuria




Is the Intracorpuscular or Extracorpuscular




Is it inherited or Acquired

Intracorpuscular defect




Aquired

Immune HA




Nonimmune HA




Is the Intracorpuscular or Extracorpuscular

Extracorpuscular

Infections, Mechanical causes, Chemical/physical agents, Acquired membrane disorder




Immune HA or NonImmune HA

NonImmune HA

Alloimmune


Autoimmune (Warm, Cold autoagglutinins)


Drug induced




Immune HA or Nonimmune HA

Immune HA

Immune hemolytic anemia has a positive or negative Coombs test

Positive

Hemogloinemia and Hemoglobinuria can be found in

Intravascular

Spherocytosis can be found in which hemolysis

Extravascular

Immune HA that occurs when the body attacks foreign things




Ex: Transfusion Rxn, Pregnancy, HDFN, and Organ transplants

Alloimmune

Immune HA where Ab attacks self AG




Ex: Warm AIHA, Cold aggultin (IgM class), Paroxysmal Cold hemoglobinuria, Drug induced, Mixed warm and cold

Autoimmune

Immune HA is DAT positive or negative

positive

In the mixed autoimmune HA:




both warm reacting Ig___ autoAb and cold reacting Ig___ autoAb




DAT detects both _____ and _____

warm= IgG




cold= IgM




DAT detects: C3 and IgG

Drug absorption (hapten) DAT is typically postivie or negative

positive

Accounts for 16-30% of AIHA cases




Usually IgG with C' activation


~most Ab react with I/H Ags




Mostly in older adults





Cold AIHA

Severity of Cold AIHA is related to

thermal range of Ab

2 Types of Cold AIHA

Idiopathic CAD




Secondary CAD

This Cold AIHA is:




usually chronic




Ab involved is usually monoclononal IgM/kappa, Autoanti-I specificity




Often linked to lymphoproliferative disorder

Idiopathic CAD

Associated with infectious disease


~50% M. pneumoniae




Secondary to lymphoproliferativve disorder


~usually chronic

Secondary CAD

Lab findings of this disease are:




Falsely decr RBC




Falsely incr MCV




Mild to moderate anemia


~normochromic/normocytic


~Polychromasia, spherocytes, clumps of RBCs


~NRBCs, erthrophagocytosis




BM: Normoblastic hyperplasia

Cold AIHA

rare autoimmune hemolytic disorder




can occur at any age




Massive intermittent acute hemolysis and hemoglobinuria




Accounts for 30-40% fo all AIHA children


~mostly under 5y

PCH

When do you do saline replacement




1:5 dilution with saline and is incubated for 30 min, then repeat the prev Cold AIHA sample

when MCHC greatly increased

Biphasic C' fixing IgG antiAb


~Donath Landsteiner antiAb


~binds to RBC at low temps --> activates C'



PCH




Paroxysmal Cold Hemoglobinuria

In PCH, what happens at 37C

Ab detaches


RBC lysed by C' activation

Lab findings:




Neutropenia, neutrophil shift to left




Reticulocytopenia, spherocytes




Incr serum bilirubin, BUN, LD




Decr C', haptoglobin




Erythrophagocytosis

PCH

Hemolytic process cuased by microcirculatory lesions that causes:


~damage to endothelial linging of the small vessels


~Deposition of platelets and fibrin in microvasulature

Hemolytic Uremic Syndrome

PT, APTT nrmal




Proteinuria, hematuria, pyuria




Incr in BUN, creatinine




hyokalemia, hypoatermia




Hemoglobinema, hemoglobinuria




Incr bilirubin, LD




Decr haptoglobin

Hemolytic Uremic Syndrome

What are disorders associated with MAHA

DIC




TTP




HUS

Mutisystem disorder


~RBC fragmentation, thrombocytopenia, acute nephropathy




2 groups: D + HUS,, D- HUS

Hemolytic Uremic Syndrome

Pathophysiology of Hemolytic Uremic syndrome

E. Coli 0157

in this group there is:




bloody diarrhea




Acute renal afilure




Chronic renal insufficiency




High in age 0-2

D+ HUS

non-diarrhea associated




seen in both children and adults




more likely to be asociated with recurring disease than the other group of Hemolytic uremic syndrome

D- HUS

compsoed of platelets and unusually large forms of VWF multimers




occlude capillaries and arterioles in organs (kidneys, heart, brain, pancreas)

Microthrombi




ass with Thrombotic Throbocytopenic Purpura

Thrombotic Thrombocytopenic Purpura has a def in _________ which is a protease that cleaves large VWF multimers

ADAMTS13

Lab findings:




Normocytic, normochromic




Polychromasia, NRBC




2+-3+ shistocytes




Leukocytosis with left shift




Sever thromobctypenia



TTP

Normal coag is altered by Bacterial sepsis, neoplasms, immunologic disorder or trama




Damage to endothelial ling of vessels


~deposition of fibrin (RBC get tangled in it)



DIC

Prologned PT, APTT, IT




Incre D-dimer, FDPs




Decr fibrinogen

DIC

associated with strnous exercise




transient hemolysis




usually no anemia




no shistocytes




Fe def can occur

Excercise-Induced Hemogloninuria


(March Hemoglobinuria)

What antagonists cause HA

Intracellular infections


~Malaria, Babesia




Extracellular infections


~CLostridiumperfringes, Bartonella bacilliformis

mild normocytic, normochromic anemia




extravascular destruction of parasitized erythrocytes


~Black water fever (massive acuteintravasuclar hemolysis)

Malarial parasites

protozoan infection (intracellular ring-like strucutes)




Extravascular hemolysis




Mild to moderate anemia




Incr retics




incr liver enzymes, bilirubin

Babesiosis

Pleomorphic organism




transmitted by blood sucking arthropods or scratch/bite from a mammal (cat)




Infected RBCs and endothelilal cells


Bartonellosis




(Bartonella bacilliformis)

Normal flora of GI tract




Transient bacteremia or life-threatining




produce potentexotoxins that affect RBC membrane




Rapid, massive, intravascular hemolysis




DIC, Neutroopenia, Thrombocytopenia, Neutropillia

Clostridium perfringens