Aggressive NK-cell leukemia
Aggressive NK-cell leukemia | |
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Synonyms | N/A |
Pronounce | N/A |
Specialty | N/A |
Symptoms | Fever, weight loss, night sweats, hepatosplenomegaly, lymphadenopathy |
Complications | Hemophagocytic lymphohistiocytosis, multi-organ failure |
Onset | Typically in young adults |
Duration | Acute |
Types | N/A |
Causes | Genetic mutations, Epstein-Barr virus |
Risks | Immunosuppression, Asian descent |
Diagnosis | Blood test, bone marrow biopsy, immunophenotyping |
Differential diagnosis | T-cell leukemia, B-cell lymphoma, infectious mononucleosis |
Prevention | N/A |
Treatment | Chemotherapy, hematopoietic stem cell transplantation |
Medication | N/A |
Prognosis | Poor |
Frequency | Rare |
Deaths | N/A |
Aggressive NK-cell leukemia | |
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ICD-10 | |
ICD-9 | |
DiseasesDB | |
MedlinePlus | |
eMedicine | |
MeSH ID |
Aggressive NK-cell leukemia is a rare and rapidly progressing form of leukemia characterized by the proliferation of natural killer cells. It is considered a subtype of large granular lymphocyte leukemia.
Signs and Symptoms
Patients with aggressive NK-cell leukemia often present with systemic symptoms such as fever, weight loss, night sweats, and fatigue. Other symptoms may include hepatosplenomegaly and lymphadenopathy.
Complications
This condition can lead to severe complications, including hemophagocytic lymphohistiocytosis (HLH), a life-threatening condition characterized by excessive immune activation and organ failure.
Causes
The exact cause of aggressive NK-cell leukemia is not well understood, but it is often associated with genetic mutations and viral infections, particularly the Epstein-Barr virus (EBV).
Diagnosis
Diagnosis typically involves a combination of blood tests, bone marrow biopsy, and immunophenotyping to identify the abnormal NK cells. Cytogenetic analysis may also be performed to detect specific genetic abnormalities.
Treatment
Treatment options are limited and often include chemotherapy and stem cell transplant. The prognosis for aggressive NK-cell leukemia is generally poor, with a high rate of relapse and resistance to treatment.
Epidemiology
Aggressive NK-cell leukemia is a rare disease, with a higher prevalence in East Asia. It affects both children and adults, with a slight male predominance.
See Also
References
External Links
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