Transcript Hemolytic Anemia Part II

Hemolytic Anemia
Part II
FJ Albert, DO, DTM&H
Hospital Medicine
Lexington Medical Center
West Columbia, SC
Associate Professor
Clinical Internal Medicine
Edward Via College of Osteopathic Medicine
Carolinas Campus
Disseminated Intravascular
Coagulation (DIC)
• A systemic process producing BOTH thrombosis
and hemorrhage
• Seen in sepsis, trauma, or malignancy
Disseminated Intravascular
Coagulation Diagnosis
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Suspect if clinical picture consistent with DIC
Thrombocytopenia
Low plasma fibrinogen level
Elevated plasma D-dimer
Elevated Fibrin Degradation Products (FDP)
Microangiopathic abnormalities on blood
smear
DIC Treatment
• Most importantly, treat the underlying disease causing
the DIC
• Commonly sepsis
• Hemodynamic support
• Bleeding patients with platelet count <50,000
– Platelet transfusion (Six pack will increase platelet count
approximately 30,000)
• Bleeding patient with fibrinogen level <50 mg/dL or
elevated PT/INR
– Cryoprecipitate replaces fibrinogen (Goal >100mg/dL)
– FFP reverses PT/INR elevation
DIC Treatment
• Treatment with intravenous heparin
generally limited to chronic DIC with
predominant thrombotic picture
– Migratory thrombophlebitis (Trousseau’s sign)
– Acral ischemia (distal extremities)
DMC
TLC
DIC
Malaria Overview
• “Mal” “aria” (“bad air”)
• Most cases, by far, occur in Africa
• Most fatal cases involve children in
sub-Saharan Africa (Plasmodium falciparum)
• World wide distribution, however
– Disease of poverty
Malaria Epidemiology
Malaria Epidemiology
Malaria Overview
• Etiology is a protozoan parasite of the Plasmodium
species
• Four main types
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Plasmodium falciparum
Plasmodium vivax
Plasmodium ovale
Plasmodium malariae
• Vector is the Anopheles (female) mosquito
• Transmitted by blood
• Incubation for all species approximately 2 weeks
– P. vivax and P. ovale can also manifest many months later
• Diagnosis typically by blood smear
Sir Ronald Ross
Nobel Prize for Medicine 1902
Sir Patrick Manson
Musculoskeletal
Malleolus
Cervical
Trachea
Feet
Malaria Symptoms
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Fever
Chills
Headache
Cough
Vomiting
Myalgias
Fatigue
Malaria Signs
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Anemia (hemolytic)
Splenomegaly
Metabolic acidosis
Hypoglycemia
Renal failure
Liver failure
Hypotension
Delirium (cerebral malaria)
Malaria Life Cycle
(1) Anopheline (female) mosquito transmits
infection (primarily bites at night)
(2) Sporozoites in mosquito saliva infect human
host
(3) Sporozoites disappear from blood in
approximately 8 hours
(4) “Successful” sporozoites enter liver cells
within 30 minutes
Malaria Life Cycle
(5) Sporozoites divide asexually within liver
cells and form into shizonts, while SOME
sporozoites become dormant as hypnozoites
within the liver
 hypnozoites specific to P. vivax and P. ovale
 schizonts=“vacuoles separate from
hepatocyte cytoplasm, containing tens of
thousands of merozoites”
Malaria Life Cycle
(6) When mature, schizont ruptures, releases
merozoites into bloodstream, and infect RBCs
(7) Some merozoites infect other RBCs
(i.e. hemolytic anemia), while others develop
into gametocytes via sexual development
(8) Gametocytes taken up by a mosquito blood
mealstomach/gutsalivary glands bites
and reinfects another human host
Malaria Life Cycle
P. Falciparum
P. Vivax
Malaria
• Plasmodium vivax rare in
West Africa
• Lack of Duffy antigen
• Parasite cannot adhere
to RBCS that lack Duffy
antigen
Malaria Prevention and Treatment
• Taking prophylaxis PROPERLY prior to arrival
as well as throughout stay within an endemic
area (NO 100% protection)
• DEET-containing insecticides
• Covered clothing
• Using permethrin treated bed nets
• Primaquine after leaving endemic area
– Hypnozoite phase of P. vivax/P. ovale
Quinine
• First discovered drug to treat P. falciparum
malaria (17th century)
• Occurs naturally in bark of cinchona tree,
native to Andes Mountains of Peru and
Bolvia
– “Suppressed fevers” when bark ingested
• Discovered by Quechua Indians and brought
to Europe by Missionary Jesuits
– First treated case in Europe in 1631 (Rome, Italy)
Quinine
• Bitter taste
• Drug of choice for malaria treatment from
1600s until 1940s
• Chloroquine replaced quinine as drug of
choice in the 1940s (more tolerable)
• Several other medication classes have since
been discovered for prophylaxis and
treatment of malaria
Quinine
• Used to treat Systemic Lupus Erythematosus
(SLE)
• “Nocturnal muscle cramp remedy”
– Muscle relaxant
– Use now much less common (FDA warning
discouraging use)
• Minute quantities remain in tonic water
Medication Options for Malaria
• Chemoprophylaxis (initiated prior to arrival
and continued throughout stay in an endemic
area)
– Chloroquine
– Mefloquine
– Doxycycline
– Atovaquone-Proguanil
Medication Options for Malaria
• Options for treatment of suspected or
confirmed disease
– Quinine
– Chloroquine
– Amodiaquine
– Sulfadoxine-Pyrimethamine (Fansidar)
– Atovaquone-Proquanil (Malarone)
– Artemisinin class of drugs
• Costly
• Black Market
Henoch-Schonlein Purpura (HSP)
• Most common systemic vasculitis of
childhood
• Usually occurs ages 3-15
– Peak at ages 4-6
• Approximately 10 pecent of cases are adults
Henoch-Schonlein Purpura (HSP)
• Clinical Manifestations
– Palpable purpura, without thrombocytopenia or
coagulopathy
– Arthralgias/arthritis
– Abdominal pain
– Renal disease
Henoch-Schonlein Purpura (HSP)
• Underlying cause is not known
• Presumed immune-mediated vasculitis
triggered by antigens
– Infections (viral or bacterial) or immunizations
• No diagnostic tests exist
Palpable Purpura in HSP
Henoch-Schonlein Purpura (HSP)
• Outpatient treatment
– Oral hydration
– Bed rest
– Symptomatic relief of abdominal and joint pain
• Inpatient treatment
– IVF
– NSAIDS (e.g. naproxen)
– Steroids (oral or IV) if NSAIDS not effective
Henoch-Schonlein Purpura (HSP)
• Excellent prognosis, overall
• Small percentage develop long term renal
disease
• Long term renal disease more common in
adults with HSP