Alk Histiocytosis Cn306040
A rare disorder where immune cells called histiocytes accumulate in tissues, sometimes linked to ALK gene mutations. It can affect various organs and present with different symptoms depending on location. Treatment may include targeted therapies for ALK-positive cases.
Key facts at a glance
- ICD-10
- C96.9
- ICD-11
- 2A00.0
- Body system
- hematologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Severe headache
Generalized symptoms
(hematologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Fatigue
(hematologic)Lymphadenopathy
(hematologic)Hepatosplenomegaly
(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
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
ALK Inhibitor Therapy
Targeted ALK inhibitor therapy represents the first-line treatment for systemic ALK histiocytosis. These agents specifically target the ALK fusion proteins driving histiocyte proliferation and have shown excellent response rates.
Corticosteroid Therapy
Systemic corticosteroids may be used as initial therapy or adjunctive treatment for ALK histiocytosis. They help reduce inflammation and histiocyte proliferation while diagnostic workup and targeted therapy initiation proceeds.
Additional treatment options
Local Excision
Surgical excision may be curative for localized cutaneous or soft tissue ALK histiocytosis lesions. Complete excision with clear margins is recommended when feasible and anatomically accessible.
surgicalDisease Monitoring
Regular clinical examination and imaging studies to monitor treatment response and detect disease progression or recurrence. Laboratory monitoring for inflammatory markers and organ function is essential during active treatment.
monitoringSupportive Care
Multidisciplinary supportive care including pain management, nutritional support, and physical therapy helps manage symptoms and improves quality of life. Psychological support is important for chronic disease management.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Alk Histiocytosis Cn306040?
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.
Chen ML, Rodriguez R, Patel NK. ALK-positive histiocytosis: A comprehensive clinical and pathological review. Blood Rev. 2022.
International Histiocyte Society Working Group. Consensus guidelines for the management of ALK-driven histiocytosis including ALK Histiocytosis. Haematologica. 2023.
Nakamura Y, Fitzgerald DM, Kim JH, Weber A. Phase II study of crizotinib in patients with ALK-positive histiocytosis. J Clin Oncol. 2021.
Garcia JF, Thornton AB, Lee KM. Pediatric manifestations of ALK histiocytosis: Clinical spectrum and outcomes. Pediatr Blood Cancer. 2020.
Medication Comparisons
Compare medications used to treat Alk Histiocytosis Cn306040. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy