Immunodeficiency 99 With Hypogammaglobulinemia And Autoimmune Cytopenias C5676971
A condition with low antibody levels and autoimmune destruction of blood cells. Both children and adults can be affected. Management includes immunoglobulin replacement and treatment for autoimmune complications.
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
Autoimmune hemolytic anemia
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hypogammaglobulinemia
(immunologic)Immune thrombocytopenia
(hematologic)Autoimmune neutropenia
(immunologic)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
Immunoglobulin Replacement
Regular immunoglobulin therapy to correct hypogammaglobulinemia and provide protective antibody coverage against encapsulated bacteria and common pathogens.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Autoimmune Cytopenia Management
Treatment of immune thrombocytopenia and autoimmune hemolytic anemia with corticosteroids, rituximab, or other immunosuppressants as needed to control cytopenias while maintaining infection defense.
Additional treatment options
Antibiotic Prophylaxis
Preventive antibiotics to reduce sinopulmonary and other bacterial infections common in hypogammaglobulinemia, often continued alongside immunoglobulin replacement therapy.
medicationIntravenous Methylprednisolone for Acute Flares
High-dose corticosteroid pulses for severe, symptomatic autoimmune cytopenias not responding to oral therapy, with careful monitoring of fluid status and glucose levels.
procedureInfection Precautions and Early Intervention
Patient education on infection prevention, prompt medical evaluation for fever, and establishment of clear action plans for managing both infections and cytopenia exacerbations.
lifestyleWhen 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 Immunodeficiency 99 With Hypogammaglobulinemia And Autoimmune Cytopenias C5676971?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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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