Lymphoproliferative Syndrome 2 C3554540
A disorder affecting immune cell regulation, increasing risk of abnormal lymphocyte growth and lymphoma. Treatment involves immune monitoring and cancer prevention.
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
Fever of unknown origin
Hemophagocytic lymphohistiocytosis
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hepatosplenomegaly
(immunologic)Severe cutaneous vasculitis
(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 therapy
Lifelong immunoglobulin replacement via IV or subcutaneous route to prevent bacterial infections in XLP2 patients who typically have hypogammaglobulinemia and impaired vaccine responses.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Allogeneic stem cell transplantation
Hematopoietic stem cell transplant from matched sibling or unrelated donor to provide functional immune cells and prevent progression of lymphoproliferative disease and hemophagocytic lymphohistiocytosis.
Additional treatment options
HLH-targeted immunotherapy
Treatment with etoposide-based or alternative protocols for hemophagocytic lymphohistiocytosis episodes, including dexamethasone and cytokine-directed therapies.
medicationAntibiotic prophylaxis
Continuous prophylactic antibiotics, particularly trimethoprim-sulfamethoxazole, to prevent Pneumocystis jirovecii pneumonia and other opportunistic infections in immunosuppressed patients.
medicationRegular surveillance for HLH and malignancy
Frequent clinical evaluation and laboratory screening for signs of HLH activation (ferritin, soluble IL-2 receptor, triglycerides) and lymphoma development, enabling early intervention.
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 Lymphoproliferative Syndrome 2 C3554540?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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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