ALTERNATE NAMES

Hepatosplenic Gamma Delta T-Cell Lymphoma; HSC

DESCRIPTION

Hepatosplenic T-cell lymphoma (HSTCL) is an extremely rare, aggressive, and very unique subtype of peripheral T-cell non-Hodgkin lymphoma (NHL). It is caused by a proliferation of cytotoxic T-cells, usually gamma delta (γδ) T-cells. In very rare occasions, the condition is caused by alpha beta (αβ) T-cells. It is characterized by rapid progression and resistance to standard chemotherapy. It typically affects the liver, spleen, and bone marrow, without involvement of lymph nodes.

HSTCL primarily affects adolescents and young adults. Males are more likely to be diagnosed than females.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: Diagnostic testing for HSTCL may include:

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  Bone marrow, spleen, or liver biopsy;

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  Imaging findings, including computed tomography (CT) and positron emission tomography (PET) scan;

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  Liver function tests; and

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  F-fluorodeoxyglucose (FDG) PET/CT.

Physical findings: Individuals with HSCTL may present with:

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  Massive hepatomegaly (liver enlargement) and/or splenomegaly (spleen enlargement);

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  “B” symptoms (unexplained fevers, drenching night sweats, and significant, unintentional weight loss);

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  Severe hemophagocytic syndrome;

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  Severe fatigue;

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  Abdominal pain;

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  Jaundice (yellowing of skin);

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  Thrombocytopenia (low platelets);

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  Anemia (low red blood cells);

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  Neutropenia (low white blood cells); and

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  Lack of lymphadenopathy.

ICD-9: 202.7x

ICD-10: C86.1; C86.10

PROGRESSION

HSTCL progresses rapidly despite aggressive treatment. Individuals with HSCTL may develop hemophagocytic lymphohistiocytosis (HLH), a rare, life-threatening syndrome of extreme, uncontrollable immune activation and hyperinflammation, leading to severe organ damage and high mortality caused by dysfunctional T-cells and macrophages failing to stop immune responses. HSTCL has a poor prognosis with an estimated 5-year overall survival of less than 15%.

TREATMENT

There is no established standard of care for treatment of HSCTL. Children and adolescents with HSTCL are often treated with adult protocols. This includes high dose chemotherapy followed by allogeneic hematopoietic stem cell transplantation (HSCT). Despite these highly intensive treatments, the prognosis for HSTCL remains poor.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Results of imaging; and

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  Biopsy reports.

Suggested Listings for Evaluation:

Meets

13.05A1

13.05C

113.05A1

113.05C

Equals