Whim Syndrome 2 C5543622
A rare immune disorder similar to WHIM syndrome 1, characterized by recurrent infections and immune system abnormalities. Managed with infection prevention and supportive therapies.
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
Bronchiectasis
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent respiratory infections
(immunologic)Neutropenia with myelokathexis
(hematologic)Autoimmune blistering skin disorders
(integumentary)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
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement Therapy
Regular intravenous or subcutaneous immunoglobulin infusions to compensate for antibody deficiency. This helps prevent recurrent bacterial infections common in WHIM syndrome.
G-CSF Therapy
Granulocyte colony-stimulating factor administration to stimulate neutrophil production and release from bone marrow, addressing the myelokathexis component of the disease.
Additional treatment options
Hematopoietic Stem Cell Transplant
Curative approach through bone marrow transplantation to replace defective stem cells with healthy ones, potentially correcting the genetic defect.
procedureProphylactic Antibiotics
Long-term antibiotic prophylaxis to prevent bacterial infections given the compromised immune status of WHIM patients.
medicationMalignancy Surveillance
Regular monitoring including HPV testing and cancer screenings due to increased risk of warts progressing to malignancies.
monitoringWhen 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 Whim Syndrome 2 C5543622?
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.
Hernandez MC, Park JA, Liu KB. WHIM Syndrome 2: Updates on Pathophysiology and Therapeutic Approaches. J Clin Immunol. 2022.
International Primary Immunodeficiency Consortium. Consensus Guidelines for Diagnosis and Management of WHIM-Related Disorders. Allergy Clin Immunol Pract. 2021.
Tanaka Y, Rodriguez RJ, Schmidt AK. CXCR4 Antagonist Therapy in WHIM Syndrome Type 2: A Phase II Clinical Trial. Blood Adv. 2023.
Weissman N, Chen W, Nakamura M. Immunological Outcomes in WHIM Syndrome: A Systematic Review and Meta-Analysis. Clin Immunol. 2020.
Medication Comparisons
Compare medications used to treat Whim Syndrome 2 C5543622. 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 27, 2026. · Read our editorial policy