Lymphomatoid granulomatosis

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Lymphomatoid granulomatosis
SpecialtyHematology and oncology

Lymphomatoid granulomatosis (LYG or LG) is a very rare

granulomatosis denotes the microscopic characteristic of the presence of granulomas with polymorphic lymphoid infiltrates and focal necrosis
within it.

LG most commonly affects middle aged people,[2] but has occasionally been observed in young people.[3] Males are found to be affected twice as often as females.[4]

Causes

Lymphomatoid granulomatosis involves malignant

immunosuppressive drugs (with case reports of methotrexate[6][7][8][9][10][11][12][13][14] and azathioprine[15][16]), infections such as HIV or chronic viral hepatitis or endogenous T cell defects.[17]

Pathophysiology

The onset of the disease results in proliferation of EBV-infected malignant

seizures, unconsciousness and death, typically followed in that order.[17]

The disease has been seen to transform to diffuse large B-cell lymphoma[18] and while LG is graded I-III based on the number of large EBV-positive B-cells, grade II and III can be considered as a variant of T-cell rich diffuse large B-cell lymphoma.[4][19]

Treatment

Treatment depends on the grade (I-III) but typically consist of cortisone,

graft versus host disease after a second transplantation 4 years later. The remaining two patients died from sepsis after the transplantation.[21]

Prognosis

The current mortality is over 60% after 5 years. However, due to hematopoietic stem cell transplantation being performed only in recent years, this number could potentially be lowered in the future. In people with CNS involvement, treatment with Interferon alpha at the US National Cancer Institute resulted in complete remission in 90% of patients.[20]

See also

  • List of cutaneous conditions

References

  1. PMID 4638966
    .
  2. PMID 25321327
    .
  3. S2CID 11047705
    .
  4. ^
    S2CID 41027533
    .
  5. S2CID 47010934
    .
  6. ^
    PMID 23733760
    .
  7. ^
    PMID 23857444
    .
  8. PMID 25130128
    .
  9. PMID 24681935
    .
  10. S2CID 26750255
    .
  11. PMID 21894776
    .
  12. PMID 19556825
    .
  13. S2CID 41605244
    .
  14. S2CID 21583991
    .
  15. PMID 25674479
    .
  16. PMID 25022612
    .
  17. ^
    S2CID 8958101
    .
  18. PMID 23694794
    .
  19. PMID 24466760
    .
  20. ^
    PMID 22814713
    .
  21. PMID 23948061
    .

External links

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