Sect 4 - Non-Immune Hemolytic Anemia

Non-Immune Hemolytic Anemia

acquired extracellular defects which shorten the life of the normal RBC

- not related to an immune response

Microorganisms

Chemicals

Mechanical trauma

3 causes of Non-Immune Hemolytic Anemia

Malaria

Babesia

Bartonella bacilliformis

Clostridium perfringens

4 microorganisms that can cause Non-Immune Hemolytic Anemia

Malaria

acquired by the bite of an infected Anopheles female mosquito

Plasmodium spp

Clinical Features of Malaria

cyclical fever (every 48hrs related to lysing of RBCs)

chills

headaches

nausea and vomiting

general malaise

hemoglobinuria ("Black Water Fever")

Lab Features of Malaria

pancytopenia:

leukopenia (decreased WBC count)

decreased HCT/Hgb/RBCs

thrombocytopenia (decreased plt count)

hemoglobinuria

Morphology of Malaria

normo/normo RBCs

malaria parasite in RBCs -- Schuffner's dots

Schuffner's dots

In Malaria, what was the name of the dots and rings characteristically noted in plasmodium falciparum?

Therapy for Malaria

hard to get rid of, but curable -- must dx correctly and treat immediately

antimalarial drugs can be administered as prophylaxis, before traveling to areas where the infection is endemic

drugs: Malarone, Doxycycline, Artemisinin, Chloroquine, Quinine, Primaquine

true

T or F:

Malaria may be misdiagnosed in the U.S. with symptoms like cold and flu

Babesiosis

a protozoan transmitted by infected ticks

Clinical Features of Babesiosis

flu-like illness with night sweats and fever

Morphology of Babesiosis

multiple organisms can be seen in cell -- maltose cross

Clindamycin, Quinine

Therapy for Babesia

Bartonella bacilliformis

caused by gram neg pleomorphic bacillus

- transmitted by sand fly "Phlebotomist Verrucurum"

- seen most Peru, Ecuador and Colombia

aka Oroya Fever or Carrion's Disease

pleomorphic

organisms that are variable in shape (cocci or rod shaped)

Clinical Features of Bartonella bacilliformis

begins with flu-like symptoms

skin lesions

secondary stage - warty tumors

lymphadenopathy

Lymphadenopathy

enlarged lymph nodes

swelling (legs) -- lymph system can't get rid of it

Morphology of Bartonella bacilliformis

organisms found on surface of RBCs

appear as purple rods, filaments or coccoid bodies attached to RBCs

antibiotics

Therapy for Bartonella bacilliformis

Clostrdium perfringens

gram positive bacilli

organism produces alpha toxins which attack the RBC membrane to produce severe hemolysis (tissue damage)

gas gangrene

Clostridium perfringens clinical infection

sepsis

Main problem of Clostridium perfringens

Lab Features of Clostridium perfringens

normo/normo RBCs

occasional microspherocytes

hemoglobinuria

increase in serum-free Hgb & decreased haptoglobin

Therapy for Clostridium perfringens

debridement of wound, very painful to pt

antibiotics, poured into wound

Arsenic

Lead

Copper

Water

Venoms

Chemical causes of hemolysis

all can cause lysis of RBCs

arsenic

inhalation of arsine gas can cause severe hemolysis of RBCs

can cause slow, prolonged, agonizing death

Clinical Features of Arsenic

severe anemia, jaundice, hemoglobinuria

nausea and vomiting

schistocytes, polychromasia, microspherocytes

Morphology of Arsenic poisoning

chelation & exchange therapy

Therapy of Arsenic poisnoning

Lead poisoning

found most often in children who have ingested lead-based paint

interference in heme production

hemolysis due to lead poisoning is caused by

true

T or F:

The affinity for lead is greater than for iron

Clinical Features of Lead Poisoning

anorexia, anemia, pica, sporadic vomiting

clumsiness, poor motor skills, convulsions

cerebral degeneration, brain damage

Morphology of Lead Poisoning

micro/hypo

cabot rings (disrupted DNA synthesis)

coarse basophilic stippling (RNA)

ring sideroblasts in bone marrow (RNA - string of pearls)

chelation therapy

Therpy for Lead poisoning

Copper Poisnoning

toxic effects are caused by inhibition of reducing enzyme systems and the glycolytic pathway of the RBC membrane causing it to lyse

1. excessive intake of copper

2. exposure to hemodialysis solution contaminated with copper

3. suicide attempts with ingestion of copper sulfate (cleaning) solutions

three ways people were exposed to copper poisoning

Clinical Features of Copper Poisoning

nausea, vomiting, abdominal pain, diarrhea

Lab Features of Copper Poisoning

polychromasia, schistocytes, spherocytes (possible)

hemoglobinemia, hemoglobinuria

water

leads to osmotic damage and lysis of cells

ex: drowning victims

venoms

poisons from bees, wasps, spider, snakes, and scorpions

similar to DIC in that poisons activate clotting factors and use them up

What does venom do to blood? What condition is it similar to?

Burns

Microangiopathic Hemolytic Anemia

Macroangiopathic Hemolytic Anemia

3 types of Mechanical Damage that causes hemolysis

burns

RBC damage by heat, cells are osmotlically and mechanically fragile

microangiopathic

tiny vessels

capillaries, arterioles and venules

TTP, HUS, DIC

3 types of Microangiopathic Hemolytic Anemias

Thrombotic Thrombocytopenic Purpura

What does TTP stand for?

TTP

condition which causes the precapillary arterioles and capillaries of the brain, kidney, heart, adrenals and pancreas to become occluded (blocked) by plt aggregates and fibrin

microangiopathic

Clinical Features of TTP

Neurological symptoms (headache, seizures, coma)

Possible renal dysfunction (proteinuria, hematuria)

Multi-organ failure

ADAMTS13 deficiency

TTP is caused by

needed to breakdown vonWillebrand's factor which is used in the adhesion process of making a clot

Why is ADAMTS13 needed?

↓ADAMTS13 = ↑ vonWillebrands = ↑ clots = ↓ plts

Lab Features of TTP

decreased plts / increased WBCs (shift to left)

schistocytes

abnormal kidney function test

deficiency of ADAMTS13 protease

Therapy for TTP

plasma exchange with FFP

anti-platelet drugs: Corticosteroids

has all the clotting factors

How does FFP work as TTP therapy?

Hemolytic Uremic Syndrome

What does HUS stand for?

HUS

involves intravascular thrombi with an associated microangiopathic blood picture

virulent E.coli (0157:H7)

HUS is usually due to toxin produced by what bacteria?

kidneys

the only organ involved in HUS

after 24hrs, HUS is irreversible

After 24 hours, is HUS reversible?

Clinical Features of HUS

acute febrile illness with nausea and vomiting followed by renal dysfunction, renal failure and hypertension

Lab Features of HUS

increased WBC

normal-decreased plts

increased retic

increased BUN and Creatinine

Burr cells, schistocytes, NRBCs

RBC, granular, and hyaline casts present

Therapy of HUS

supportive with blood transfusion

hemodialysis

Disseminated Intravascular Coagulation

What does DIC stand for?

DIC

principle event is due to trauma which activates the coagulation cascade leading to intravascular generation of thrombi (clots)

intravascular thrombi

blood clots that form inside a blood vessel or the heart chambers

Malignancy

Obstretic complications

Septicimia

Trauma

What causes DIC (MOST)?

Lab Features of DIC

decreased plts

schistocytes and burr cells

coag studies

decreased clotting factors = increased PT & APTT

increased bleeding time

positive DDimer

Therapy for DIC

heparin - stops clotting = stops bleeding

antithrombin

maintain blood volume

heparin will maintain blood volume needs antithrombin III in order to work

What is the purpose of using heparin for therapy in DIC? What additional factor does heparin need to work?

Macroangiopathic Hemolytic Anemia

abnormalities within the heart and large vessels that may cause hemolysis of RBCs

stents - lead to tissue damage = clots

any other foreign objects in the body

March Hemoglobinuria

Hypersplenism

Lipid Disorders

Liver Disease

Renal Disease

5 Other Non-Immune HA Causes

March Hemoglobinuria

a mile hemolytic disorder that occurs with repetitive traumatic stress exerted to the palms of the hands and soles of the feet

ex: soldiers, runner, bongo drummers, etc

Hypersplenism

RBC survival is shortened by enlarged spleen

the larger the spleen, the longer it takes the RBC to come out alive

Lipid Disorders

changes in the blood lipid concentration cause abnormalities in the RBCs membrane lipid composition, abetalipoproteinemia

acanthocytes

For lipid disorders, what was the morphology is seen on peripheral blood smear?

cirrhosis

What condition of the liver can cause hemolysis?

Renal Disease

retained excretory products in patients with renal disease creates unfavorable conditions for RBC

burr cells

What is the RBC morphology related to renal disease?

they are smaller, so it takes less of a bacterial load to get them sick

Why are children more susceptible than adults to bacteria?