ALLERGY & IMMUNOLOGY TiKi TaKa

. HYPER-SENSITIVITY REACTIONS:

{1} Type "1" (IMMEDIATE):

. Ex. Acute atopic dermatitis.
. Highly pruritic papules, vesicles & plaques.
. Light microscopy -> Spongiosis (edema of the epidermis).

{2} Type "2" (ANTIBODY MEDIATED):

. IgM or IgG + ANTIGEN.
. Ex. Immune hemolytic anemia & Rh hemolytic disease of the newborn.

{3} Type "3" (IMMUNE COMPLEX MEDIATED):

. Ag + Ab + COMPLEMENT.
. Ex. Serum sickness.

{4} Type "4" (CELL MEDIATED):

. Dermal inflammation after direct contact with allergen.
. Ex. Tuberculin skin test & Allergic contact dermatitis.

. TRANSFUSION REACTIONS:
. 1. ABO INCOMPATIBILITY:
. Acute symptoms of hemolysis WHILE the transfusion is occurring.
. Ex -> DURING a transfusion, the pt becomes hypotensive & tachycardic.
. Back & chest pain & dark urine.
. ++ LDH & bilirubin.
. -- Haptoglobin.

. 2. TRANSFUSION RELATED ACUTE LUNG INJURY (TRALI) = LEUKO-AGGLUTINATION REACTION:

. Acute Shortness of breath from antibodies in the donor blood against the recipient WBCs.
. Ex: 20 mins after a pt. receives a blood transfusion, the pt becomes short of breath.
. Transient infiltrates on CXR.
. All symptoms resolve spontaneously.

. 3. IgA DEFECIENCY:
. presents with anaphylaxis !
. In the future, use blood donations from an IgA deficient donor or washed RBCs.
. Ex -> As soon as the pt. received transfusion, he becomes SOB, hypotensive & tachycardic.
. NORMAL LDH & BILIRUBIN.

. 4 . MINOR BLOOD GROUP INCOMPATIBILITY:

. To kell, Duffy, Lewis or Kidd antigens or Rh incompatibility !
. Delayed jaundice.
. Ex -> A few days after transfusion, the pt becomes jaundiced.
. The hematocrit doesn't rise with transfusion & he is generally without symptoms: . No specific therapy.

. 5. FEBRILE NON-HEMOLYTIC REACTION:

. Small rise in temperature - No ttt required.
. Reaction against donor WBCs antigens.
. prevented by using filtered blood transfusions in the future to remove WBCs antigens.
. Ex -> A few hours after transfusion, the pt becomes febrile with rise 1 degree in temp.
. No evidence of hemolysis.

. RHINITIS:
{A} ALLERGIC RHINITIS:
. Watery rhinorrhea & sneezing with more prominent eye symptoms.
. Early age of onset.
. Identifiable trigger (animals - environmental exposure).
. Usually seasonal symptoms but can be persistent throughout year.
. Nasal mucosa can be normal, pale blue or pale on exam.
. Associated with allergic disorders e.g. eczema & asthma.
. Tx -> Allergen avoidance.
. Tx -> Topical intra-nasal glucocorticoids.

{B} NON-ALLERGIC RHINITIS = VASOMOTOR RHINITIS:

. Nasal congestion - Rhinorrhea - Postnasal discharge (postnasal drip = dry cough).
. Late age of onset > 20 ys.
. Can't identify clear trigger !
. Symptoms throughout the year but sometimes worse with seasons change.
. Nasal mucosa may be normal or erythematous.
. Less commonly associated with allergic disorders e.g. asthma or eczema.
. Routine allergy testing isn't necessary prior to initiating empiric ttt.
. May respond to 1st generation oral H1 antihistaminics (Chloramphenicol),
. Never responds to antihistaminics without anticholinergic properties (Loratidine)!
. Tx -> TOPICAL INTRANASAL GLUCOCORTICOIDS.

. The 3 most common causes of CHRONIC COUGH (> 8 weeks):

. BRONCHIAL ASTHMA.
. GERD.
. UPPER AIRWAY COUGH $YNDROME = POST-NASAL DRIP:
. NON-smoker.
. Caused by rhino-sinusitis conditions.
. Dry cough is most likely due to post-nasal drip associated with allergic rhinitis.
. Dx -> Confirmed by improvement of the nasal discharge & cough with H1 Anti-histaminics.
. Chlorpheniramine is an H1 receptor blocker that decreases the allergic response.
. Decrease in NASAL SECRETIONS is most likely to significally improve symptoms.

. ALLERGIC REACTIONS:
{1} . ANAPHYLAXIS = ANAPHYLACTIC SHOCK:
. Type 1 hypersensitivity reaction.
. Pts usually have prior exposure to the offending substance.
. Pts have pre-formed Ig E -> Histamine mediated peripheral vasodilatation.
. Bee stings - food & medications are the most common allergens.
. Acute onset of hypotension & tachycardia.
. Dangerous allergic reaction may progress to respiratory failure & circulatory collapse.
. Allergen exposure -> Sudden onset of symptoms in more than one system,
. Cutaneous (hives - flushing - pruritis).
. GIT (Lip / tongue swelling - vomiting).
. Respiratory (Dyspnea - wheezing - stridor - hypoxia).
. Cardiovascular (Hypotension).
. It is a medical emergency.
. Tx -> INTRA-MUSCULAR EPINEPHRINE into the THIGH.

{2} . ANGIO-EDEMA:
. H/O of ICU pt on ACEIs e.g ENALAPRIL.
. Edema in the face, mouth, lips.
. Absence of pruritis & urticaria.
. Laryngeal edema may occur causing airway obstruction.
. occurs due to BRADYKININ release.
. it may occur at any time not just at the start of drug intake.
. Dx----> Low levels of C2 & C4.
. Tx----> STOP ACEIs + FRESH FROZEN PLASMA + Secure the airway.

. HERIDITARY angioedema:
. C1 esterase inhibitor deficiency.
. usually follows an infection, dental procedure or minor trauma.
. N.B. The most common cause of acquired isolated angioedema is ACE inhibitors use.

. N.B. C1q is NORMAL in hereditary angioedema.

. N.B. C1q is DEPRESSED in acquired angioedema.

. C4 levels are depressed in all forms !

{3} . URTICARIA:
. Sudden swellings of the superficial layers of the skin.
. Can be caused by insects or medications.
. May be caused by pressure, cold or vibration !
. Tx -> ANTI-HISTAMINICs (Diphenhydramine & koratidine).

. GRAFT VERSUS HOST DISEASE (GVHD):

. in pts with bone marrow transplantation.
. due to activation of the DONOR "T" lymphocytes.
. Skin ---> Maculopapular rash.
. Intestine ---> Bloody diarrhea.
. Liver ---> Abnormal LFTs & jaundice.

. PRIMARY IMMUNO-DEFECIENCY DISORDERS:

{1} COMMON VARIABLE IMMUNODEFECIENCY (CVID):
. ADULT with recurrent sino-pulmonary infections.
. NORMAL NUMBER OF "B" cells but don't make effective amounts of immunoglobulins.
. DECREASE in ALL subtypes of immunoglobulins (IgG, IgM & IgA).
. Frequent episodes of bronchitis, pneumonia, sinusitis & otitis media.
. CVID increases the risk of lymphoma.
. Dx -> Decreased immunoglobulins level & -- response to Ag stimulation of B-cells.
. Tx -> Antibiotics for infections.
. Tx -> Chronic maintenance with regular infusions of I.V. immunoglobulins.
. The clue to CVID is DECREASE in the OUTPUT of B-LYMPHOCYTES with,
. NORMAL NUMBER of B-cells & NORMAL amount of LYMPHOID TISSUE (LNs, adenoids & tonsils).

{2} X-LINKED (BRUTON) A-GAMMA-GLOBULINEMIA:

. MALE CHILDREN with recurrent sino-pulmonary infections.
. DIMINISHED B-cells & LYMPHOID TISSUES.
. ABSENCE of tonsils, adenoids & lymph nodes & spleen.
. NORMAL T-cells.
. Tx -> Antibiotics for infections.
. Tx -> Chronic maintenance with regular infusions of I.V. immunoglobulins.

{3} SEVERE COMBINED IMMUNODEFECIENCY:

. Combined = Deficiency in BOTH B & T cells.
. -- B-cells -> -- immunoglobulin production -> Recurrent sinopulmonary infections at 6ms
. -- T-cells -> AIDS related infections e.g. PCP, varicella & candida.
. Treat the infections as they rise.
. BM transplantation is curative.

{4} Ig"A" DEFECIENCY:

. Recurrent sinopulmonary infections + ATOPIC DISEASE + ANAPHYLAXIS to blood transfusions
. Anaphylaxis from blood transfusions from pts with "NORMAL" levels of IgA !
. Treat infections as they arise.
. ONLY use blood that is from Ig-A DEFECIENT donors or that has been WASHED !
. IVIG will NOT work as the amount of IgA in the product is too small to be therapeutic !
. The trace amounts of IgA in IVIG may provoke anaphylaxis !

{5} HYPER Ig"E" $YNDROME:

. Recurrent SKIN infections with STAPHYLOCOCCI.
. Treat infections as they arise.
. Consider prophylactic antibiotics e.g Dicloxacillin & cephalexin.

{6} WISKOTT - ALDRICH $YNDROME:

. IMMUNODEFECIENCY + THROMBOCYTOPENIA + ECZEMA.
. MARKED DECREASED T-LYMPHOCYTES.
. BM TRANSPLANTATION is the ONLY curative ttt.

{7} CHRONIC GRANULOMATOUS DISEASE (CGD):

. Genetic disease results in extensive inflammatory reactions.
. Lymph nodes with purulent material leaking out !
. Aphthous ulcers & inflammation of the nares.
. Obstructive granulomas in the GIT or UT.
. Infections with odd combinations (Staphylococci, Bulkhorderia, Nocardia & Aspergillus).
. Dx -> ABNORMAL TETRAZOLIUM TEST !
. -- in respiratory burst that produces hydrogen peroxide.
. -- in NADPH oxidase that generates superoxide.

Dr. Wael Tawfic Mohamed