_ Absolute: significant aspirin allergy

Clopidogrel
Side effects: bleeding, rash, rare neutropenia

t present, if patients have an elevated transferrin saturation or ferritin level,

genetic testing should be performed; if they are a C282Y homozygote or a compound
heterozygote (C282Y/H63D), the diagnosis is confirmed. If liver enzymes (alanine
aminotransferase [ALT], aspartate aminotransferase [AST]) are elevated or the ferritin is
>1000 g/L, the patient should be considered for liver biopsy because there is an increased
frequency of advanced fibrosis in these individuals. If liver biopsy is performed, iron
deposition is found in a periportal distribution with a periportal to pericentral gradient; iron is
found predominantly in parenchymal cells, and Kupffer cells are spared.
Contraindications

_ Absolute: active pathological bleeding (ICH, PUD)

Heparin
Side effects: bleeding, thrombocytopenia
Contraindications
_ Absolute: severe thrombocytopenia
Avoid anticoagulation and GPllb/llla inhibitors
Avoid non-steroidal agents
Treatment for chest pain (does not respond to nitroglycerin)
Bed rest (similar to acute MI)
Monitor and treat for arrhythmias

specific therapy
n/a

follow-up
n/a

complications and prognosis

Ventricular aneurysm
Late rupture

CARDIAC TUMORS
CARDIAC TUMORS
PRISCILLA HSUE, MD

history & physical

Primary Cardiac Tumors 75% benign, remainder are malignant
Benign:
Atrial myxoma
_ most common primary cardiac

_ Absolute: significant aspirin allergy

Clopidogrel
Side effects: bleeding, rash, rare neutropenia

t present, if patients have an elevated transferrin saturation or ferritin level,

genetic testing should be performed; if they are a C282Y homozygote or a compound
heterozygote (C282Y/H63D), the diagnosis is confirmed. If liver enzymes (alanine
aminotransferase [ALT], aspartate aminotransferase [AST]) are elevated or the ferritin is
>1000 g/L, the patient should be considered for liver biopsy because there is an increased
frequency of advanced fibrosis in these individuals. If liver biopsy is performed, iron
deposition is found in a periportal distribution with a periportal to pericentral gradient; iron is
found predominantly in parenchymal cells, and Kupffer cells are spared.
Contraindications

_ Absolute: active pathological bleeding (ICH, PUD)

Heparin
Side effects: bleeding, thrombocytopenia
Contraindications
_ Absolute: severe thrombocytopenia

Avoid anticoagulation and GPllb/llla inhibitors

Avoid non-steroidal agents
Treatment for chest pain (does not respond to nitroglycerin)
Bed rest (similar to acute MI)

Monitor and treat for arrhythmias

specific therapy
n/a

follow-up
n/a

complications and prognosis

Ventricular aneurysm
Late rupture

CARDIAC TUMORS
PRISCILLA HSUE, MD

history & physical

Primary Cardiac Tumors 75% benign, remainder are malignant
Benign:
Atrial myxoma
_ most common primary cardiac

_ Absolute: significant aspirin allergy

Clopidogrel
Side effects: bleeding, rash, rare neutropenia
Contraindications
_ Absolute: active pathological bleeding (ICH, PUD)
Heparin
Side effects: bleeding, thrombocytopenia
undertake diagnostic studies.
Early infusion of factor after any bleed reduces morbidity.
Joint bleeds do not require aspiration unless pain and swelling are
severe or unless sepsis is suspected.

specific therapy
Management of acute bleeding episodes in severe hemophilia

Assess whether bleed is life-threatening (intracranial, retroperitoneal.

retropharyngeal with compromise of airway, major
trauma, major surgery), major (severe joint or soft tissue bleed,
severe trauma without evidence of bleed, GI bleeding), or minor
(most joint and soft tissue bleeds, epistaxis, dental bleeding).
Life-threatening bleeds, replace to 80100%; major, replace to
50%; minor, to 30%
Clotting factor concentrates dosed in units (U) with 1 U defined
as amount of Factor VIII or Factor IX in 1 ml of normal plasma.
To calculate FVIII dose, assume that 1 U/kg raises circulating
level by 2%. To dose 70-kg man to 100% level will require 70
(kg) 50 = 3500 U, and to dose him to 30% will require 70
15 = 1050 U. To calculate FIX dose, assume that 1 U/kg raises
circulating level by 1%, so to dose 70-kg man to 100% requires 70
100 = 7,000 U. Doses for recombinant FIX (Benefix) require
Hemophilia A and B 675
an increase of 2030% because of reduced recovery. CRITICAL
TO CHECK FVIII/FIX LEVELS AFTER DOSING AND TO ADJUST
DOSE ACCORDINGLY!
Duration of treatment. For uncomplicated minor bleeds, 23
doses given q12h suffice. For major or life-threatening bleeds,
treatment must be continued for 710 days.
Both plasma-derived and recombinant products are available.
With current viral inactivation techniques, plasma-derived products
are generally safe fromHIV and hepatitis, but avoid infusing
plasma-derived product into patients who have previously been
treated only with recombinant product.
FOR MILD HEMOPHILIA, MAY BE POSSIBLE TO MANAGE
BLEEDS WITH DDAVP, SYNTHETIC ANALOGUE OF VASOPRESSIN.
CONSULTAHEMATOLOGISTBEFOREGIVINGCLOTTING
FACTOR CONCENTRATES TO A PATIENT WHO HAS
NEVER RECEIVED THEM.
Long-termmanagement of disease
Multiple studies demonstrate that 3 weekly prophylactic infusion
of 2540 U/kg of FVIII concentrate, begun at 12 yrs of age,
prevents life-threatening bleeds and greatly improves joint disease
in childrenwith hemophilia.Manypatientsnowmaintained
on such a regimen.
For those still treated in response to bleeds, not prophylactically,
early factor infusion in response to bleeds is critical for best outcome.

For chronic synovitis/anthropathy, options include intensive

physical therapy, surgical or radioactive synovectomy, or joint

replacement.
Side Effects and Complications
Failure to infuse adequate dose of factor leads to poor control of
bleeding and resulting tissue damage.
Current major complication of therapy is development of inhibitory