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- Neutrophil-Specific Granule Deficiency
- Complement Studies
Myeloperoxidase deficiency50% have complete loss / no chlorine formation in azurophilic (primary) granules / usually asymptomatic except in diabetics who have increased risk of disseminated candidiasis Chediak-Higashi Autosomal recessive mutation in LYST (microtubule and lysosomal defects) Recurrent staph and strep, partial albinism, mental retardation, platelet dysfunction, severe periodontal disease, and in those patients surviving into 20s, striking peripheral nerve defects (nystagmus, neuropathy)
neutropenia, splenomegaly, from long standing RA Complement deficiencies [labs] most are recessive, all occur at similar rates (except C2 may be more common, 1% prevalence) / C3 (severe disease) / C5-8 GC meningitis, arthritis Biology of Complement [activation cascade] Functions: lysis, opsonization, anaphylatoxins (degranulation), chemotaxis Classical: Ag:Ab complex, C1, C4, C2 attachment, activation, amplification, attack Alternative: microbe + P, D, B, C3b Lectin (new): MBP opsonizes foreign carbohydrates C3a, C5a also anaphylatoxins C5a is also a chemotactic factor
Complement Studies
C1 inhibitor (acquired or AD) hereditary angioedema / may occur in SLE, lymphoproliferative disorders, paraproteinemias recurrent GI attacks of colic are common / no pruritis or urticarial lesions Allergy
Most common B-lactams / ⅓ of cases are idiopathic 5-60 minutes following exposure, but delayed reaction is possible Angioedema with or without urticaria (not true anaphylaxis without life threatening hypotension or laryngeal edema) Presentation: pruritis, flushing, urticaria, angioedema, diaphoresis, sneezing, rhinorrhea, congestion, hoarseness, stridor, laryngeal edema, dyspnea, tachypnea, wheezing, bronchorrhea, cyanosis, tachycardia, bradycardia, hypotension, cardiac arrest, arrhythmias, nausea, vomiting, diarrhea, abdominal cramping, dizziness, weakness, syncope, sense of impending doom, seizures Treatment: Epinephrine(EpiPen, Adrenaline) IM (anterolateral thigh is fastest absorbed) Benadryl 50 mg PO or IV every 4 hrs H2 blockers (Zantac, Pepcid, etc.) Methylprednisolone (Solu-Medrol, Depo-Medrol) Recumbent position, elevate legs, oxygen (Beta2-adrenergic Agonists: Terbutaline) Volume replacement, pressors as needed Longer Term Treatments Anabolic steroids: Stanozolol (Winstrol) / increases C1 esterase inhibitor and C4 Antigonadotropic agents: Danazol (Danocrine) / increases C4 levels Serine Proteinase Inhibitors (serpins) / C1 inhibitor, human (Cinryze) / IV infusion repeated every few days Ddx: EM minor (urticarial or bullous lesions), SJS, TEN [these syndromes cause fever, headache, malaise, arthralgia, corneal ulcerations, arrhythmia, pericarditis, electrolyte abnormalities, seizures, coma, sepsis] Dilantin hypersensitivity: very common, ranges from minor to life-threatening, mechanism unclear Iodine allergy: not true allergic reaction; hyperosmolar dye causes degranulation of mast cells/basophils Pretreatment protocol: 40 mg prednisone 24 hrs before then 12 hrs, etc… / H2 blockers (ranitidine), benadryl / avoid contrast dye with renal insufficiency and sickle cell disease / normal maximum dye load would be 2 CT scans within a 24 hour period (assuming normal renal function) Download 3.95 Mb. Share with your friends:
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