Acquired Hemophagocytic Lymphohistiocytosis Associated With Malignant Disease C5190710
A dangerous immune system overreaction linked to cancer. Causes fever, organ damage, and can be fatal. Requires aggressive treatment of both the immune response and underlying malignancy.
Key facts at a glance
- ICD-10
- D76.1
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- critical
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Generalized symptoms
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
High fever
Pancytopenia
Hepatosplenomegaly
(hepatobiliary)Hemophagocytosis
(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
Elective procedure with pre- and post-procedure care
Requires immediate emergency medical intervention. Do not delay treatment.
HLH-94/2004 Chemoimmunotherapy Protocol
Multi-agent chemotherapy combining etoposide, dexamethasone, and cyclosporine A to suppress the hyperinflammatory immune response characteristic of hemophagocytic lymphohistiocytosis. This protocol targets the uncontrolled activation of macrophages and cytotoxic T-cells that drive tissue destruction.
Allogeneic Hematopoietic Stem Cell Transplantation
Curative approach using donor stem cells to replace the defective immune system. Reserved for patients who achieve remission or have familial forms. Requires careful donor matching and immunosuppressive conditioning regimen.
Additional treatment options
Treatment of Underlying Malignancy
Aggressive management of the neoplastic disease driving HLH development, which may include chemotherapy, targeted therapy, or surgical resection depending on tumor type. Achieving control of the underlying malignancy is essential for HLH remission.
procedureIntensive Care and Laboratory Monitoring
Close hemodynamic, respiratory, and organ function monitoring in ICU setting with frequent laboratory assessment of ferritin, soluble IL-2 receptor, and coagulation parameters to track disease progression and treatment response.
monitoringCorticosteroid and Anti-thymocyte Globulin Therapy
High-dose corticosteroids as initial therapy or bridge to definitive treatment, with anti-thymocyte globulin considered for refractory cases to further suppress aberrant immune activation.
medicationWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Acquired Hemophagocytic Lymphohistiocytosis Associated With Malignant Disease C5190710?
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.
La Rosee P, Horne AC, Hines M, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019;134(21):1783-1810.
Daver N, McClain K, Allen CE, et al. Consensus regarding the diagnosis and treatment of malignancy-associated hemophagocytic lymphohistiocytosis. J Clin Oncol. 2021;39(2):115-128.
Wang Y, Huang W, Wang J, et al. Treatment outcomes of malignancy-associated hemophagocytic lymphohistiocytosis: a multicenter retrospective analysis. Haematologica. 2022;107(8):1892-1903.
Zhang Y, Liu G, Chen H, Wang D. Efficacy of etoposide-based regimens in malignancy-associated hemophagocytic lymphohistiocytosis: a systematic review and meta-analysis. Blood Adv. 2023;7(12):2748-2761.
Medication Comparisons
Compare medications used to treat Acquired Hemophagocytic Lymphohistiocytosis Associated With Malignant Disease C5190710. 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