Tags

Type your tag names separated by a space and hit enter

[Multisystemic amyloidosis. Clinical study of 39 patients in Lebanon].
Presse Med. 2005 May 14; 34(9):640-6.PM

Abstract

OBJECTIVE

Clinical analysis of multisystem amyloidosis in Lebanon, by histological type.

METHOD

Retrospective study of 39 cases of multisystem amyloidosis diagnosed histologically in a university hospital center between 1991 and 2002. It analyzed the following clinical data: age, gender, type of presentation, time from symptom onset to diagnosis, clinical features, concomitant diseases, family history of amyloidosis, biopsy sites, presence of urinary or serum monoclonal gammopathy, immunohistochemical type, prognosis and treatment.

RESULTS

Median age at diagnosis was 56+/-18 years. The overall ratio of men to woman was 1.4. AL amyloidosis (amyloid light chain) accounted for 54% (21/39) of the cases, AA (amyloid-associated) amyloidosis 36% (14/39), while 10% (4/39) were not typed. Among the 21 cases of AL amyloidosis, 12 were idiopathic (57%) and 9 (43%) were associated with multiple myeloma; among the 14 cases of AA amyloidosis, 7 were associated with familial Mediterranean fever and 5 with chronic disorders. Proteinuria was often the first symptom. The initial manifestations in AL amyloidosis patients with myeloma were more often related to amyloidosis than to myeloma. Renal involvement was seen in 95% (37/39) of all cases (95% of AL versus 93% of AA), proteinuria in 87% of cases and renal failure in 72%. Cardiac amyloidosis (57% of AL versus 7% of AA; p>0.05), infiltration of the tongue (19% of AL versus 0% of AA; p>0.05) and neurological manifestations (24% of AL versus 7% of AA; p>0.05) were more frequent in AL amyloidosis. The 7 patients who died (18%) had AL amyloidosis (5 of them had myeloma). Heart failure was the most frequent cause of death related to amyloid.

CONCLUSION

Multisystem amyloidosis is frequent in Lebanon and familial Mediterranean fever is still frequently associated with the secondary type. Accurate diagnosis and classification are essential for the prognosis and treatment of the disease. Poor prognosis was associated with the AL type, especially when accompanied by myeloma, and with cardiac amyloidosis.

Authors+Show Affiliations

Service de médecine interne, Hôtel-Dieu de France, Université Saint Joseph, Faculté de médecine, Beyrouth, Liban.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

English Abstract
Journal Article
Review

Language

fre

PubMed ID

15988337

Citation

Saba, Mona, et al. "[Multisystemic Amyloidosis. Clinical Study of 39 Patients in Lebanon]." Presse Medicale (Paris, France : 1983), vol. 34, no. 9, 2005, pp. 640-6.
Saba M, Tohmé A, Abadjian G, et al. [Multisystemic amyloidosis. Clinical study of 39 patients in Lebanon]. Presse Med. 2005;34(9):640-6.
Saba, M., Tohmé, A., Abadjian, G., Haddad, F., & Ghayad, E. (2005). [Multisystemic amyloidosis. Clinical study of 39 patients in Lebanon]. Presse Medicale (Paris, France : 1983), 34(9), 640-6.
Saba M, et al. [Multisystemic Amyloidosis. Clinical Study of 39 Patients in Lebanon]. Presse Med. 2005 May 14;34(9):640-6. PubMed PMID: 15988337.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Multisystemic amyloidosis. Clinical study of 39 patients in Lebanon]. AU - Saba,Mona, AU - Tohmé,Aline, AU - Abadjian,Gérard, AU - Haddad,Fady, AU - Ghayad,Elie, PY - 2005/7/1/pubmed PY - 2005/7/21/medline PY - 2005/7/1/entrez SP - 640 EP - 6 JF - Presse medicale (Paris, France : 1983) JO - Presse Med VL - 34 IS - 9 N2 - OBJECTIVE: Clinical analysis of multisystem amyloidosis in Lebanon, by histological type. METHOD: Retrospective study of 39 cases of multisystem amyloidosis diagnosed histologically in a university hospital center between 1991 and 2002. It analyzed the following clinical data: age, gender, type of presentation, time from symptom onset to diagnosis, clinical features, concomitant diseases, family history of amyloidosis, biopsy sites, presence of urinary or serum monoclonal gammopathy, immunohistochemical type, prognosis and treatment. RESULTS: Median age at diagnosis was 56+/-18 years. The overall ratio of men to woman was 1.4. AL amyloidosis (amyloid light chain) accounted for 54% (21/39) of the cases, AA (amyloid-associated) amyloidosis 36% (14/39), while 10% (4/39) were not typed. Among the 21 cases of AL amyloidosis, 12 were idiopathic (57%) and 9 (43%) were associated with multiple myeloma; among the 14 cases of AA amyloidosis, 7 were associated with familial Mediterranean fever and 5 with chronic disorders. Proteinuria was often the first symptom. The initial manifestations in AL amyloidosis patients with myeloma were more often related to amyloidosis than to myeloma. Renal involvement was seen in 95% (37/39) of all cases (95% of AL versus 93% of AA), proteinuria in 87% of cases and renal failure in 72%. Cardiac amyloidosis (57% of AL versus 7% of AA; p>0.05), infiltration of the tongue (19% of AL versus 0% of AA; p>0.05) and neurological manifestations (24% of AL versus 7% of AA; p>0.05) were more frequent in AL amyloidosis. The 7 patients who died (18%) had AL amyloidosis (5 of them had myeloma). Heart failure was the most frequent cause of death related to amyloid. CONCLUSION: Multisystem amyloidosis is frequent in Lebanon and familial Mediterranean fever is still frequently associated with the secondary type. Accurate diagnosis and classification are essential for the prognosis and treatment of the disease. Poor prognosis was associated with the AL type, especially when accompanied by myeloma, and with cardiac amyloidosis. SN - 0755-4982 UR - https://www.unboundmedicine.com/medline/citation/15988337/[Multisystemic_amyloidosis__Clinical_study_of_39_patients_in_Lebanon]_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0755-4982(05)84002-3 DB - PRIME DP - Unbound Medicine ER -