eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology
Severe Combined Immunodeficiency: Treatment & Medication
Updated: Aug 18, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
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Treatment
Medical Care
Conventional care for any patient with severe combined immunodeficiency (SCID) includes isolation to avoid infection and meticulous skin and mucosal hygienic care while awaiting stem cell reconstitution. Signs of sepsis and pulmonary infections may be subtle; fever mandates a detailed search for infectious agents. Empiric broad-spectrum antibiotics should be administered parenterally while awaiting the results of cultures and body fluid analysis. Consider prophylactic treatment with nystatin to prevent mucocutaneous candidiasis.In individual cases, prophylaxis with antiviral agents such as acyclovir or antibiotics, also may be appropriate. Parenteral nutrition is customarily provided to children with diarrhea and failure to thrive. Ancillary therapy includes intravenous immunoglobulin replacement. Live vaccines should not be used. Blood product transfusions must be lymphocyte-depleted and irradiated to prevent transfusion-associated graft versus host disease (GVHD).
- Bone marrow or other stem cell reconstitution is the first-line emergent therapy specific for almost all forms of severe combined immunodeficiency. With early transplantation and aggressive monitoring and treatment of infections, survival rates may be as high as 97%.
- In the largest series of patients with severe combined immunodeficiency, bone marrow transplantation (BMT) was successful in 80% of patients. T-cell function has been adequate in approximately 90% of patients who survive 6 months posttransplant, and B-cell function has been adequate in 70% of these patients. Workup includes major histocompatibility complex (MHC) typing to identify a fully matched sibling, or, in the case of consanguinity, possibly a parent. Reconstitution using a matched unrelated donor or haploidentical parent also have been successful, although GVHD occurs more frequently in these recipients. The lack of functional T cells in patients with severe combined immunodeficiency obviates the need for pretransplant myeloablative chemotherapy, thus reducing the toxicity of the procedure. Pretransplant evaluation routinely includes testing of the recipient and the donor for infectious agents, such as cytomegalovirus (CMV), HIV, and hepatitis.
- In utero BMT into the fetal peritoneal cavity is successful, with reconstitution of T-cells in X-linked (XL) severe combined immunodeficiency and in one case of severe combined immunodeficiency due to IL-7R α-chain deficiency.
- Cord blood stem cell transplantation from related or unrelated donors is an option.
- Gene therapy is a viable option in patients with XL severe combined immunodeficiency or adenosine deaminase (ADA) deficiency severe combined immunodeficiency who have no human leukocyte antigen (HLA)-identical sibling. Treatment is optimally given prior to age 4 months to reduce the risks of failed gene transduction and leukemia. Gene therapy is also predicted to work for JAK3 and RAG2 mutations based on murine studies.
- ADA deficiency was the first form of severe combined immunodeficiency for which gene therapy was attempted, and efficacy has been reported in 4 patients.
- In 1999, a clinical trial for XL severe combined immunodeficiency gene therapy began, and data suggest that in cases of successful gene insertion, functional T cells developed within 18 weeks and were detectable as long as 5 years later. Adverse events have included failure of gene insertion and acute lymphoblastic leukemia due to aberrant insertion within the LMO-2 gene, both of which occurred in older patients. Other studies have confirmed the risk for leukemia in patients who underwent gene therapy and attempts are underway to minimize it.4
- Specific therapy for dermatitis and eosinophilia in severe combined immunodeficiency is immunosuppression with cyclosporine and possible addition of interferon (IFN)-γ. These modalities have been used to treat Omenn syndrome but theoretically should be effective in treating maternal or transfusion-induced GVHD.
Surgical Care
- Surgical intervention is customarily not indicated.
Consultations
- Laboratory studies for stem cell reconstitution must be initiated promptly with the BMT team. In the meantime, gastroenterology and nutrition consultations provide important support.
Diet
- The presence of chronic diarrhea and failure to thrive requires consultation with gastroenterology and nutrition.
- Parenteral or enteral nutritional supplementation is often necessary to ensure adequate intake of calories, nutrients, and vitamins.
Activity
- Infants with any form of severe combined immunodeficiency are isolated to decrease the risk of common viral and bacterial infections. Avoidance of crowds in such places as stores, doctors' offices, and hospitals is important, along with customary hygiene practices, like strict handwashing.
- The earlier practice of putting patients in reverse isolation ("bubble") with such precautions as special diets is no longer advocated.
Medication
First-line therapy for severe combined immunodeficiency (SCID) is allogeneic hematopoietic stem cell transplantation. The optimal bone marrow donor is a human leukocyte antigen (HLA)–matched sibling or parent if consanguinity is present. Haploidentical parent donors, HLA-matched unrelated donors, and HLA 5/6 allele–matched unrelated donors have also been successful; however, the risk for graft failure, graft versus host disease (GVHD), and inadequate B-cell function is higher.
Aggressive therapy for suspected or proven infection is essential. Antibiotic coverage typically must be broad-spectrum. Antiviral agents include acyclovir, foscarnet, or ganciclovir for varicella-zoster virus (VZV), herpes simplex virus, and cytomegalovirus (CMV). Antifungal therapy includes fluconazole for mucocutaneous candidiasis; amphotericin B is first-line therapy for invasive fungal infections such as Aspergillus.
Nutritional support is imperative because undernutrition decreases the success rate for stem cell reconstitution and increases the risk for opportunistic infections.
X-linked (XL) severe combined immunodeficiency and adenosine deaminase (ADA) deficiency may alternatively be treated with gene therapy. Polyethylene glycol–treated (PEG) ADA replacement may be administered, with improvement but not complete reconstitution of immune function.
Replacement therapy with intravenous immunoglobulin (IVIG) in patients with primary immune deficienciesThe overall consensus among clinical immunologists is that a dose of IVIG of 400-600 mg/kg/mo or a dose that maintains trough serum immunoglobulin (Ig)G levels greater than 500 mg/dL is desirable. Patients with X-linked agammaglobulinemia and meningoencephalitis require much higher doses (1 g/kg) and perhaps intrathecal therapy. Measurement of preinfusion (trough) serum IgG levels every 3 months until a steady state is achieved and then every 6 months if the patient is stable may be helpful in adjusting the dose of IVIG to achieve adequate serum levels. For persons who have a high catabolism of infused IgG, more frequent infusions (eg, every 2-3 wk) of smaller doses may maintain the serum level in the reference range. The rate of elimination of IgG may be higher during a period of active infection; measuring serum IgG levels and adjusting to higher dosages or shorter intervals may be required.
For replacement therapy for patients with primary immune deficiency, all brands of IVIG are probably equivalent, although differences exist in viral inactivation processes (eg, solvent detergent vs pasteurization and liquid vs lyophilized). The choice of brands may be dependent on the hospital or home care formulary and the local availability and cost. The dose, manufacturer, and lot number should be recorded for each infusion in order to review for adverse events or other consequences. Recording all side effects that occur during the infusion is crucial.
Monitoring liver and renal function test results periodically, approximately 3-4 times a year, is also recommended. The US Food and Drug Administration (FDA) recommends that for patients at risk for renal failure (eg, those with preexisting renal insufficiency, diabetes, volume depletion, sepsis, paraproteinemia, those >65 y, and those who use nephrotoxic drugs) recommended doses should not be exceeded and infusion rates and concentrations should be the minimum levels that are practicable.
The initial treatment should be administered under the close supervision of experienced personnel. The risk of adverse reactions in the initial treatments is high, especially in patients with infections and those who form immune complexes. In patients with active infection, infusion rates may need to be slower and the dose halved (ie, 200-300 mg/kg), with the remaining dose given the next day to achieve a full dose. Treatment should not be discontinued. After achieving normal serum IgG levels, adverse reactions are uncommon unless patients have active infections.
With the new generation of IVIG products, adverse effects are greatly reduced. Adverse effects include tachycardia, chest tightness, back pain, arthralgia, myalgia, hypertension or hypotension, headache, pruritus, rash, and low-grade fever. More serious reactions are dyspnea, nausea, vomiting, circulatory collapse, and loss of consciousness. Patients with profound immunodeficiency or patients with active infections have more severe reactions.
Anticomplementary activity of IgG aggregates in the IVIG and the formation of immune complexes are thought to be related to the adverse reactions. The formation of oligomeric or polymeric IgG complexes that interact with Fc receptors and trigger the release of inflammatory mediators is another cause. Most adverse reactions are rate related. Slowing the infusion rate or discontinuing therapy until symptoms subside may diminish the reaction. Pretreatment with ibuprofen (5-10 mg/kg orally [PO] every 6-8 h), acetaminophen (15 mg/kg/dose PO), diphenhydramine (1 mg/kg/dose PO), and/or hydrocortisone (6 mg/kg/dose, not to exceed 100 mg) 1 hour before the infusion may prevent adverse reactions. In some patients with a history of severe side effects, analgesics and antihistamines may be repeated.
Acute renal failure is a rare but significant complication of IVIG treatment. Reports suggest that IVIG products using sucrose as a stabilizer may be associated with a greater risk for this renal complication. Acute tubular necrosis, vacuolar degeneration, and osmotic nephrosis are suggestive of osmotic injury to the proximal renal tubules. The infusion rate for sucrose-containing IVIG should not exceed 3 mg sucrose/kg/min. Risk factors for this adverse reaction include preexisting renal insufficiency, diabetes mellitus, dehydration, age older than 65 years, sepsis, paraproteinemia, and concomitant use of nephrotoxic agents. For patients at increased risk, monitoring BUN and creatinine levels before starting the treatment and prior to each infusion is necessary. If renal function deteriorates, the product should be discontinued.
IgE antibodies to IgA have been reported to cause severe transfusion reactions in IgA-deficient patients. A few reports exist of true anaphylaxis in patients with selective IgA deficiency and common variable immunodeficiency who developed IgE antibodies to IgA after treatment with immunoglobulin. In actual experience, however, this is very rare. In addition, this is not a problem for patients with X-linked agammaglobulinemia (Bruton disease) or severe combined immunodeficiency. Caution should be exercised in those patients with IgA deficiency (<7 mg/dL) who need IVIG because of IgG subclass deficiencies. IVIG preparations with very low concentrations of contaminating IgA are advised (see Table 2).
Table 2. Immune Globulin, Intravenous5,6,7,8
Open table in new window
Table
| Brand(Manufacturer) | Manufacturing Process | pH | Additives* | Parenteral Form and Final Concentrations | IgA Content mcg/mL |
| Carimune NF (CSL Behring) | Kistler-Nitschmann fractionation; pH 4, nanofiltration | 6.4-6.8 | 6% solution: 10% sucrose, <20 mg NaCl/g protein | Lyophilized powder 3%, 6%, 9%, 12% | Trace |
| Flebogamma (Grifols USA) | Cohn-Oncley fractionation, PEG precipitation, ion-exchange chromatography, pasteurization | 5.1-6 | Sucrose free, contains 5% D-sorbitol | Liquid 5% | <50 |
| Gammagard Liquid 10% (Baxter Bioscience) | Cohn-Oncley cold ethanol fractionation, cation and anion exchange chromatography, solvent detergent treated, nanofiltration, low pH incubation | 4.6-5.1 | 0.25 M glycine | Ready-for-use liquid 10% | 37 |
| Gamunex (Talecris Biotherapeutics) | Cohn-Oncley fractionation, caprylate-chromatography purification, cloth and depth filtration, low pH incubation | 4-4.5 | Contains no sugar, contains glycine | Liquid 10% | 46 |
| Iveegam EN (Baxter Bioscience) | Cohn-Oncley fraction II/III; ultrafiltration; pasteurization | 6.4-7.2 | 5% solution: 5% glucose, 0.3% NaCl | Lyophilized powder 5% | <10 |
| Polygam S/D Gammagard S/D (Baxter Bioscience for the American Red Cross) | Cohn-Oncley cold ethanol fractionation, followed by ultracentrafiltration and ion exchange chromatography; solvent detergent treated | 6.4-7.2 | 5% solution: 0.3% albumin, 2.25% glycine, 2% glucose | Lyophilized powder 5%, 10% | <1.6 (5% solution) |
| Octagam (Octapharma USA) | Cohn-Oncley fraction II/III;ultrafiltration; low pH incubation; S/D treatment pasteurization | 5.1-6 | 10% maltose | Liquid 5% | 200 |
| Panglobulin (Swiss Red Cross for the American Red Cross) | Kistler-Nitschmann fractionation; pH 4, trace pepsin, nanofiltration | 6.6 | Per gram of IgG: 1.67 g sucrose, <20 mg NaCl | Lyophilized powder 3%, 6%, 9%, 12% | 720 |
| Privigen Liquid 10% (CSL Behring) | Cold ethanol fractionation, octanoic acid fractionation, and anion exchange chromatography; pH4 incubation and depth filtration | 4.6-5 | L-proline (~250 mmol/L) as stabilizer; trace sodium; does not contain carbohydrate stabilizers (eg, sucrose, maltose) | Ready-for-use liquid 10% | <25 |
| Brand(Manufacturer) | Manufacturing Process | pH | Additives* | Parenteral Form and Final Concentrations | IgA Content mcg/mL |
| Carimune NF (CSL Behring) | Kistler-Nitschmann fractionation; pH 4, nanofiltration | 6.4-6.8 | 6% solution: 10% sucrose, <20 mg NaCl/g protein | Lyophilized powder 3%, 6%, 9%, 12% | Trace |
| Flebogamma (Grifols USA) | Cohn-Oncley fractionation, PEG precipitation, ion-exchange chromatography, pasteurization | 5.1-6 | Sucrose free, contains 5% D-sorbitol | Liquid 5% | <50 |
| Gammagard Liquid 10% (Baxter Bioscience) | Cohn-Oncley cold ethanol fractionation, cation and anion exchange chromatography, solvent detergent treated, nanofiltration, low pH incubation | 4.6-5.1 | 0.25 M glycine | Ready-for-use liquid 10% | 37 |
| Gamunex (Talecris Biotherapeutics) | Cohn-Oncley fractionation, caprylate-chromatography purification, cloth and depth filtration, low pH incubation | 4-4.5 | Contains no sugar, contains glycine | Liquid 10% | 46 |
| Iveegam EN (Baxter Bioscience) | Cohn-Oncley fraction II/III; ultrafiltration; pasteurization | 6.4-7.2 | 5% solution: 5% glucose, 0.3% NaCl | Lyophilized powder 5% | <10 |
| Polygam S/D Gammagard S/D (Baxter Bioscience for the American Red Cross) | Cohn-Oncley cold ethanol fractionation, followed by ultracentrafiltration and ion exchange chromatography; solvent detergent treated | 6.4-7.2 | 5% solution: 0.3% albumin, 2.25% glycine, 2% glucose | Lyophilized powder 5%, 10% | <1.6 (5% solution) |
| Octagam (Octapharma USA) | Cohn-Oncley fraction II/III;ultrafiltration; low pH incubation; S/D treatment pasteurization | 5.1-6 | 10% maltose | Liquid 5% | 200 |
| Panglobulin (Swiss Red Cross for the American Red Cross) | Kistler-Nitschmann fractionation; pH 4, trace pepsin, nanofiltration | 6.6 | Per gram of IgG: 1.67 g sucrose, <20 mg NaCl | Lyophilized powder 3%, 6%, 9%, 12% | 720 |
| Privigen Liquid 10% (CSL Behring) | Cold ethanol fractionation, octanoic acid fractionation, and anion exchange chromatography; pH4 incubation and depth filtration | 4.6-5 | L-proline (~250 mmol/L) as stabilizer; trace sodium; does not contain carbohydrate stabilizers (eg, sucrose, maltose) | Ready-for-use liquid 10% | <25 |
*IVIG products containing sucrose are more often associated with renal dysfunction, acute renal failure, and osmotic nephrosis, particularly with preexisting risk factors (eg, history of renal insufficiency, diabetes mellitus, age >65 y, dehydration, sepsis, paraproteinemia, nephrotoxic drugs).
Enzyme replacement
Improved immune function and clinical response are observed with PEG-ADA replacement for ADA deficiency.
Pegademase bovine (Adagen)
Modification of ADA by PEG conjugation of bovine ADA increases the half-life of the enzyme and reduces the immunogenicity of the protein.
Adult
30 U/kg IBW IM twice weekly
Pediatric
Administer as in adults
Pentostatin decrease effect of pegademase bovine; vidarabine is a substrate for ADA and may alter effect
Theoretical allergic reaction to foreign protein; severe thrombocytopenia
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Possible need for higher doses in younger children, who clear enzyme more rapidly; adjust until patient clinically stable with improved immune functions (lymphocyte count and proliferative responses to mitogens in vitro); therapeutic ADA levels have been established by measuring trough levels
Antiviral agents
Herpes simplex virus, CMV, and VZV are treated with acyclovir. PO absorption is poor; thus, most patients require IV administration. Ganciclovir is an alternative drug, also administered IV, for the same viral infections. Both drugs are used for prophylaxis after exposure to VZV beyond the 72- to 96-hour period within which VZIG is effective at 50% of the therapeutic dose.
Acyclovir (Zovirax)
High dose of 45-60 mg/kg/d, or 1500 mg/m2/d divided q8h is used for CNS infection. Good hydration is essential, and lower doses must be calculated in the presence of renal compromise.
Adult
1500 mg/m2/d IV divided q8h for 10-14 d
Pediatric
Administer as in adults
Concomitant use of probenecid or zidovudine prolongs half-life and increases CNS toxicity of acyclovir
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Lower doses with renal impairment; caution with premature infants; poor hydration increases risk for precipitation in renal tubules; headaches, encephalopathy, GI irritation, rashes, arthralgias, fever, and bone marrow suppression
Ganciclovir (Cytovene)
DOC for CMV and is used for HSV and VZV resistant to acyclovir.
Adult
Therapy: 10 mg/kg/d IV divided q12h for 14-21 d
Maintenance: 5-6 mg/kg/d IV for 5-7 d/wk; infuse IV over 1 h or longer
Prevention: 5-6 mg/kg/dose IV qd for 5-7 d/wk; alternatively, 1000 mg PO tid with food (PO absorption is poor)
Pediatric
>3 months: Administer as in adults for treatment; IV infusion is over 1 h or longer
Prevention: 5 mg/kg IV qd
Concomitant administration with cytotoxic drug (eg, dapsone, vinblastine, Adriamycin, pentamidine, flucytosine, vincristine, amphotericin B, trimethoprim/sulfamethoxazole, nucleoside analogs) may result in additive toxicity in bone marrow, spermatogonia, and germinal layers of skin and GI mucosa (coadminister only if potential benefits outweigh risks)
Coadministration with imipenem-cilastatin may cause generalized seizures (use only if potential benefits outweigh risks); serum creatinine level may increase following concurrent use of ganciclovir with either cyclosporine or amphotericin B; in presence of probenecid, ganciclovir renal clearance is reduced; bioavailability may increase when didanosine is administered either 2 h before or simultaneously with ganciclovir; bioavailability of ganciclovir may decrease in presence of zidovudine, while bioavailability of zidovudine is increased in presence of ganciclovir
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Lower dosage with renal impairment; neutropenia, thrombocytopenia, confusion, and retinal detachment; reconstituted solutions of IV ganciclovir have a high pH (11); phlebitis or pain may occur at site of IV infusion, despite further dilution in IV fluids; administration of ganciclovir should be accompanied by adequate hydration; photosensitization (photoallergy or phototoxicity) may occur
Antifungal agents
Mucocutaneous candidiasis usually can be treated with fluconazole. Invasive Candida, Aspergillus, and other fungal infections require IV amphotericin B. Prevention of Aspergillus infection and treatment of certain Candida resistant to fluconazole may be performed effectively with itraconazole.
Fluconazole (Diflucan)
Fungistatic activity. Synthetic PO antifungal (broad-spectrum bistriazole) that selectively inhibits fungal CYP450 and sterol C-14 alpha-demethylation, which prevents conversion of lanosterol to ergosterol, thereby disrupting cellular membranes. Requires a loading dose on day 1 followed by maintenance at 50% of the loading dose. May be administered by either IV or PO routes with similar efficacy. Length of treatment is a minimum of 10 d; longer courses are determined individually, considering other risk factors such as ongoing broad-spectrum antibiotics.
Adult
Loading dose: 400 mg PO/IV followed by 200 mg PO/IV qd
Pediatric
Loading dose: 10 mg/kg PO/IV followed by 3-6 mg/kg PO/IV qd
Levels may increase with thiazide diuretics; fluconazole levels may decrease with long-term coadministration of rifampin; coadministration of fluconazole may decrease phenytoin clearance; fluconazole is a potent inhibitor of CYP450 3A isoenzyme and may increase concentrations of theophylline, tolbutamide, glyburide, and glipizide; effects of anticoagulants may increase with fluconazole coadministration; increases in cyclosporine concentrations may occur when administered concurrently
Documented hypersensitivity; cardiac arrhythmias may occur with cisapride, terfenadine, and astemizole
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Adjust dose for renal insufficiency; monitor closely if rashes develop and discontinue drug if lesions progress; may cause clinical hepatitis, cholestasis, and fulminant hepatic failure (including death) with underlying medical conditions (eg, AIDS, malignancy) and while taking multiple concomitant medications; not recommended for breastfeeding mothers
Itraconazole (Sporanox)
Used most commonly to prevent Aspergillus infection. PO solution, 10 mg/mL, is administered on an empty stomach; capsules, 100 mg, are taken with food.
Adult
600 mg/24 h PO divided tid for 3-4 d; followed by 400 mg/d PO divided bid; in severe cases, initial high dose is continued for longer period
Pediatric
5-10 mg/kg/d PO qd or divided bid
Antacids may reduce absorption of itraconazole; CYP450 3A isoenzyme inhibitor; edema may occur with coadministration of calcium channel blockers (eg, amlodipine, nifedipine); hypoglycemia may occur with sulfonylureas; may increase tacrolimus and cyclosporine plasma concentrations when high doses are used; rhabdomyolysis may occur with coadministration of HMG-CoA reductase inhibitors (lovastatin or simvastatin); coadministration with cisapride can cause cardiac rhythm abnormalities and death
May increase digoxin levels; coadministration may increase plasma levels of midazolam or triazolam; phenytoin and rifampin may reduce itraconazole levels (phenytoin metabolism may be altered)
Documented hypersensitivity; coadministration with cisapride may cause adverse cardiovascular effects (possibly death)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in hepatic insufficiencies; GI symptoms, headaches, rash, and hypokalemia
Amphotericin B (Amphocin, Fungizone)
Test dose of 0.1 mg/kg is recommended by manufacturer but often omitted. Infusion of total dose over 2-4 h has been recommended, but infusion over 1 h seems to be adequate. Because of the high incidence of toxicity, renal, hepatic, electrolyte, and hematologic status must be monitored closely. In particular, potassium and magnesium levels usually are monitored daily. Salt loading with 10-15 mL/kg of NS before each dose is used to decrease the risk of nephrotoxicity. Premedication with acetaminophen and diphenhydramine 30 min before and 4 h after infusion decreases the typical adverse effects of fever, chills, hypotension, nausea and vomiting. Hydrocortisone may be admixed to IV (1 mg/mg amphotericin, not to exceed 25 mg).
Adult
1 mg/kg/d or 1.5 mg/kg qod IV
Pediatric
Administer as in adults
Antineoplastic agents may enhance the potential of amphotericin B for renal toxicity, bronchospasm, and hypotension; corticosteroids, digitalis, and thiazides may potentiate hypokalemia; risk of renal toxicity is increased with cyclosporine
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Monitor renal function, serum electrolytes (eg, magnesium, potassium), liver function, CBC count, and hemoglobin concentrations; resume therapy at lowest level (eg, 0.25 mg/kg) when therapy is interrupted for more than 7 d
Lipid amphotericin B products
Three amphotericin products are available: amphotericin B lipid complex (Abelcet), amphotericin B cholesteryl sulfate (Amphotec), and amphotericin B liposomal (AmBisome). Lipid amphotericin B is used when toxicity from nonlipid amphotericin B is unacceptable. In some patients, lipid products seem to cause less fever, GI irritation, chills, and headache. Not clear whether renal toxicity is lower.
Adult
3-5 mg/kg/d infused IV over 2 h
Pediatric
Administer as in adults
Antineoplastic agents may enhance the potential of amphotericin B for renal toxicity, bronchospasm, and hypotension; corticosteroids, digitalis, and thiazides may potentiate hypokalemia; risk of renal toxicity is increased with cyclosporine
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Premedicate with acetaminophen and diphenhydramine; monitoring of renal, electrolyte, hepatic, and hematologic status essential
More on Severe Combined Immunodeficiency |
| Overview: Severe Combined Immunodeficiency |
| Differential Diagnoses & Workup: Severe Combined Immunodeficiency |
 Treatment & Medication: Severe Combined Immunodeficiency |
| Follow-up: Severe Combined Immunodeficiency |
| Multimedia: Severe Combined Immunodeficiency |
| References |
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Further Reading
Keywords
severe combined immunodeficiency, SCID, X-linked SCID, XL-SCID, MHC class II deficiency, bare lymphocyte syndrome, adenosine deaminase–deficient SCID, ADA-deficient SCID, recurrent infections, failure to thrive, dermatitis, bone marrow transplantation, DiGeorge syndrome, CHARGE syndrome, hematopoietic stem cell transplantation, HSCT, otitis media, cytomegalovirus infection, CMV, varicella, respiratory syncytial virus, RSV, rotavirus, parainfluenza virus, Epstein-Barr virus, EBV, enterovirus, adenovirus, non-Hodgkin lymphoma, herpes simplex virus, cryptosporidiosis, Crohn disease, HIV infection, graft versus host disease, GVHD, Omenn syndrome, treatment, diagnosis
