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Home > G. Tumoral pathology > ALK+ anaplastic large cell lymphoma

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ALK+ anaplastic large cell lymphoma

t(2;5)(p23;q35) creating NPM1/ALK fusion protein

Synopsis

- 2%-7% of non-Hodgkin lymphomas

Immunochemistry

- CD30+
- EMA+ (50%)
- P53+

Variants

- monomorphic ALCL
- small cell ALCL
- lymphohistiocytic ALCL
- Hodgkin-like ALCL
- sarcomatoid ALCL
- neutrophil-rich ALCL
- eosinophil-rich ALCL
- signet-ring ALCL

Associations

- HIV infection

Differential diagnosis

- classic Hodgkin lymphoma

  • syncitial variant of sclerosing nodular Hodgkin lymphoma
  • lymphocyte depletion Hodkgkin lymphoma

- sinusoidal CD30-positive large B-cell lymphoma (10757332)
- HHV-8-associated lymphoma (15105661)
- Carcinomas
- melanoma

Molecular biology

- fusion genes invoving the ALK gene

  • NPM1/ALK fusion protein (70-80%) by t(2;5)(p23;q35)
  • TPM3/ALK fusion protein (20%) by t(1;2)(q25;p23)
  • MSN/ALK fusion protein by t(X;22)(q11;p23)
  • ATIC/ALK fusion protein by inv(2)(p23q35)
  • TFG/ALK fusion protein by t(2;3)(p23;q21)
  • CLTC/ALK fusion protein by t(2;17)(p23;q23)
  • ALO17/ALK fusion protein by t(2;17)(p23;q25)
  • MYH9/ALK fusion protein t(2;22)(p23;q11.2)

See also

- cutaneous CD30+ anaplastic large-cell lymphoma (cutaneous CD30+ ALCL)

References

- Vassallo J, Lamant L, Brugieres L, Gaillard F, Campo E, Brousset P, Delsol G. ALK-positive anaplastic large cell lymphoma mimicking nodular sclerosis Hodgkin’s lymphoma: report of 10 cases.Am J Surg Pathol. 2006 Feb;30(2):223-9. PMID: 16434897

- Allory Y, Merabet Z, Copie-Bergman C, Lange F, Yiou R, Gaulard P. Sarcomatoid variant of anaplastic large cell lymphoma mimics ALK-1-positive inflammatory myofibroblastic tumor in bladder. Am J Surg Pathol. 2005 Jun;29(6):838-9; PMID: 15897755

- Nakamura S, Shiota M, Nakagawa A, Yatabe Y, Kojima M, Motoori T, Suzuki R, Kagami Y, Ogura M, Morishima Y, Mizoguchi Y, Okamoto M, Seto M, Koshikawa T, Mori S, Suchi T. Anaplastic large cell lymphoma: a distinct molecular pathologic entity: a reappraisal with special reference to p80(NPM/ALK) expression. Am J Surg Pathol. 1997 Dec;21(12):1420-32. PMID: 9414185

P.S.

- AGCOH