X Linked Dyserythropoetic Anemia With Abnormal Platelets And Neutropenia C3550856
A rare genetic blood disorder affecting red blood cell development, platelet function, and neutrophil counts. Primarily affects males. Treatment involves blood transfusions, infection prevention, and monitoring by a hematologist.
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.
Warning signs - seek immediate care
Severe infections
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Neutropenia
(hematologic)Anemia
(hematologic)Thrombocytopenia
(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
Blood Transfusion Support
Red blood cell transfusions to manage symptomatic anemia, with frequency determined by transfusion-dependent needs and iron status.
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Comprehensive Hematologic Monitoring
Regular monitoring of blood counts including hemoglobin, platelet levels, and neutrophil counts with regular hematology review.
Additional treatment options
Iron Chelation Therapy
Management of iron overload secondary to chronic transfusions using iron chelating agents to prevent end-organ damage.
medicationInfection Prophylaxis
Antibiotic prophylaxis and prompt treatment of infections given the inherent neutropenia and associated infection risk.
medicationNutritional Support and Folate Supplementation
Dietary counseling to ensure adequate nutrition including folate supplementation to support erythropoiesis in chronic anemia.
dietaryWhen 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 X Linked Dyserythropoetic Anemia With Abnormal Platelets And Neutropenia C3550856?
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.
Thompson AA, Williams DA. Inherited bone marrow failure syndromes: pathophysiology and clinical approaches. Hematology Am Soc Hematol Educ Program. 2022.
Alter BP, Rogers SC, Meyn MS. Diagnosis and management of inherited bone marrow failure syndromes: Guidelines from the National Marrow Failure Gene Study. Blood Adv. 2021.
Lichtenstein JR, Freedman MH, Gross S. X-linked dyserythropoietic anemia with neutropenia and thrombocytopenia: Clinical and molecular features. Blood. 2018.
Germing U, Hofmann JM, Strupp C. Clinical manifestations and long-term outcomes in congenital dyserythropoietic anemias: A multicenter retrospective analysis. Haematologica. 2020.
Medication 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