Lymphomatoid granulomatosis natural history

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kamal Akbar, M.D.[2]

Overview

Prognosis is generally variable and the 5-year mortality rate of patients with Lymphmatoid granulomatosis is approximately 63-90%. In many instances there is totally remission of the disease

Natural History, Complications, and Prognosis

Natural History

The symptoms of Lymphomatoid granulomatosis usually develop in the fifth to the sixth decade of life, and start with symptoms such as Cough, Dyspnea , and Chest tightness. Patient mostly present with pulmonary symptoms but in 40-50% of cases patients will present with cutaneous symptoms and then 30 present of patients will present with CNS symptoms.^[1]

Complications

The most common complication of the disease can be Lymphoma. In some cases the disease does progress to lymphoma if patient is not cared for.^[2]^[3]

Prognosis

Depending on the extent of the disease at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor.^[4]

References

↑ Messana, Kate; Marburger, Trent; Bergfeld, Wilma (2015). “EBV-Negative Cutaneous Lymphomatoid Granulomatosis With Concomitant EBV-Positive Pulmonary Involvement”. The American Journal of Dermatopathology. 37 (9): 707–711. doi:10.1097/DAD.0000000000000198. ISSN 0193-1091.
↑ Grimm, Kate E.; O’Malley, Dennis P. (2019). “Aggressive B cell lymphomas in the 2017 revised WHO classification of tumors of hematopoietic and lymphoid tissues”. Annals of Diagnostic Pathology. 38: 6–10. doi:10.1016/j.anndiagpath.2018.09.014. ISSN 1092-9134.
↑ Song JY, Pittaluga S, Dunleavy K, Grant N, White T, Jiang L; et al. (2015). “Lymphomatoid granulomatosis–a single institute experience: pathologic findings and clinical correlations”. Am J Surg Pathol. 39 (2): 141–56. doi:10.1097/PAS.0000000000000328. PMC 4293220. PMID 25321327.
↑ Halvani A, Owlia MB, Sami R (2010). “Lymphomatoid granulomatosis with splenomegaly and pancytopenia”. Zhongguo Fei Ai Za Zhi. 13 (1): 84–6. doi:10.3779/j.issn.1009-3419.2010.01.17. PMC 6000673. PMID 20672711.

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[MessanaMarburger2015-1] Messana, Kate; Marburger, Trent; Bergfeld, Wilma (2015). “EBV-Negative Cutaneous Lymphomatoid Granulomatosis With Concomitant EBV-Positive Pulmonary Involvement”. The American Journal of Dermatopathology. 37 (9): 707–711. doi:10.1097/DAD.0000000000000198. ISSN 0193-1091.

[GrimmO'Malley2019-2] Grimm, Kate E.; O’Malley, Dennis P. (2019). “Aggressive B cell lymphomas in the 2017 revised WHO classification of tumors of hematopoietic and lymphoid tissues”. Annals of Diagnostic Pathology. 38: 6–10. doi:10.1016/j.anndiagpath.2018.09.014. ISSN 1092-9134.

[pmid25321327-3] Song JY, Pittaluga S, Dunleavy K, Grant N, White T, Jiang L; et al. (2015). “Lymphomatoid granulomatosis–a single institute experience: pathologic findings and clinical correlations”. Am J Surg Pathol. 39 (2): 141–56. doi:10.1097/PAS.0000000000000328. PMC 4293220. PMID 25321327.

[pmid20672711-4] Halvani A, Owlia MB, Sami R (2010). “Lymphomatoid granulomatosis with splenomegaly and pancytopenia”. Zhongguo Fei Ai Za Zhi. 13 (1): 84–6. doi:10.3779/j.issn.1009-3419.2010.01.17. PMC 6000673. PMID 20672711.

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