Castleman Kojima Disease C4552543
A rare condition involving abnormal growth of lymph node tissue, often with systemic symptoms like fever and fatigue. It can affect both children and adults and may be linked to other health conditions. Treatment depends on the specific type and may include medications or other therapies.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized lymphadenopathy
(hematologic)Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Fever
(hematologic)Night sweats
(hematologic)Unexplained weight loss
(hematologic)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
Corticosteroid therapy
High-dose corticosteroids such as prednisone to control inflammatory component of Castleman-Kojima disease. Often serves as first-line systemic therapy.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Anti-IL-6 therapy (tocilizumab)
Monoclonal antibody targeting interleukin-6 receptor to address the underlying inflammatory cascade characteristic of TAFRO syndrome variant.
Additional treatment options
Surgical excision of localized lesions
Complete surgical resection of unicentric Castleman disease lesions when localized and surgically accessible, potentially curative.
surgicalImmunosuppressive agents
Agents such as cyclosporine or cyclophosphamide may be used for refractory cases not responding to steroids or anti-IL-6 therapy.
medicationMultidisciplinary monitoring
Regular monitoring of hematologic parameters, renal function, and imaging to assess treatment response and detect complications such as Castleman-associated infections or malignancy.
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 Castleman Kojima Disease C4552543?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Castleman Kojima Disease C4552543. 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