Lymphomatoid granulomatosis natural history
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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.