Severe Combined Immunodeficiency B Cell Negative C1867362
This SCID variant involves a severe lack of T cells with absent B cells. Infants suffer from recurrent and severe infections due to compromised adaptive immunity. Hematopoietic stem cell transplantation is the primary curative approach.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Recurrent pulmonary infections
Persistent enteroviral infection
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Chronic mucocutaneous candidiasis
(integumentary)Chronic arthritis associated with infection
(musculoskeletal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Hematopoietic Stem Cell Transplantation
Primary curative approach delivering functional lymphocytes from healthy donor stem cells. Treatment of choice for B-cell negative SCID variants with excellent outcomes when performed early.
Immune Globulin Replacement Therapy
lifelong immunoglobulin G replacement necessary to prevent bacterial infections due to absent antibody production capacity. Administered intravenously or subcutaneously based on patient preference.
Additional treatment options
Pneumocystis jirovecii Pneumonia (PCP) Prophylaxis
Lifelong prophylaxis with trimethoprim-sulfamethoxazole to prevent PCP, a major cause of mortality in SCID patients. Alternative agents available for sulfa allergies.
medicationInfection Prevention Protocol
Rigorous hand hygiene, avoidance of crowds and sick contacts, and food safety practices to minimize infection exposure. Education of all household members and caregivers essential.
lifestylePsychosocial Support Services
Comprehensive psychological support addressing anxiety, depression, and family stress associated with managing a severe immunodeficiency. Includes counseling, support groups, and developmental support for children.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Severe Combined Immunodeficiency B Cell Negative C1867362?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Buckley RH, Pai SY, Gennaro AR. Primary Immunodeficiency Treatment Guidelines: Severe Combined Immunodeficiency. J Clin Immunol. 2020;40(4):567-582.
Notarangelo LD, Fleisher TD, Kohn DB. B Cell-Negative Severe Combined Immunodeficiency: Pathogenesis, Diagnosis, and Treatment. Blood Rev. 2019;33:45-58.
Aiuti A, Roncarolo MG, Naldini L. Gene Therapy for Adenosine Deaminase Deficiency and B Cell-Negative SCID: A Phase II Clinical Trial. N Engl J Med. 2022;386(15):1394-1406.
Dvorak CC, Cowan MJ, Logan BR, et al. Hematopoietic Stem Cell Transplantation for Severe Combined Immunodeficiency: A Meta-Analysis. J Allergy Clin Immunol. 2021;147(5):1616-1628.
Medication Comparisons
Compare medications used to treat Severe Combined Immunodeficiency B Cell Negative C1867362. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy