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The prognostic significance of paraproteinaemia in rheumatoid arthritis
404 THE PROGNOSTIC PARAPROTEINAEMIA Kelley
CA, Baird
G:,
SlCNIFICANCE OF IN RHEUMATOID Foster H.. Griffiths
Departments of Rheumatology Newcastle upon Tyne
ARTIIRITIS
ID.
and Haematology*.
RVI,
The incidence of Ivm~homa and mveloma are increased in rheumatoid tmhti~is (RA), alth&g’b the reasons kz this arr incompletely nndrrs!wd. We have assessed the predictive value of monoclonal nammo”arhy in RA for the later devcloomeni of lvmohooroliferativc maiienanc’v. A serum paraprorei” was discbvered in 23 p&&s (14 female) wiih .s“&ian age of 59 years and a media” duratlo” of RA of 6 years. The “araurotei” was quantified and funher investigations including a bone m&w &aminatio” and skeletal survey were undenake” to investigate the possible development of a lymphoproliferative disorder. Secondary Sjogren’s Syndrome (SSS) war noted in 12 patients. The pamprotein was moneclonal in 21 patients and was classed as IgG (12 cases). IgA (6 cases) or IgM (3 cases). Biclonal tmraorotcins were see” in 2 w&e”nrs. Free light chains were found in the ;ri”i of 8 patients. Bone%w~ow ab”o&nlttier were found in 13 individuals. I” two thirds of oatients with either an IeA or a” IeM paraprotei” the bane marrow wa; abnormal, whereas less thin half of thise with a” IgG pamprotein had a” abnormal marrow. Patients were followed Up for a media” of 4 years and five patients with a” abnormal bone marrow developed myeloma of whom 3 had a” IgAh paraprotein. Two other patients developed non-Hodgkins lymphoma. Exua-articular bony erosions Were in 4 oatients. three of whom develooed mveloma but the “txsence of urinary f;ee light chains was not sp’ecific’for the dev&pment of malignancy. Paraprotcinsemra I” RA indicacs monoclonal B cell proliferation and carries an increased risk of malignant trasformario”.
AFTER SURAMIN THERAPY, A METASTATIC ADRENAL CARCINOMA SWITCHED FROM PRODUCING ALDOSTERONE TO PRODUCING CORTISOL Ciompi ML’. *Rheumatology
Amaw L*. De Giorgio F.‘. Barn&i L.*. Mmemini R.” Unit and “Department of Surgery-Urology Unit. University
of Piss.
CASE REPORT. Hypertension and hypokalemia appeared in a fifty-years old woman in 19% On treatment with anti-hypertensive drags, she consulted our Rheumatology Unit complaining muscle weakness at her legs and fatigue. Clinical chemistry showed hypokalemia, hypcmaamia, alkaline urine pH, and a primary aldosteronism was supposed. lncmase in plasma aldostemnc coneentratio” (619 pg/ml) and B-scan images ~ontirmd diagnosis. Plasma renin. corurol and A(;TH levela were in normal values. Opratrve tntewe.ntt~n with a&ton of the tumor was ptrformed in Februv 1991. and the hlsmlagical examination showed a macro-adenoma with a “CC~OOCarea inside. No enlargement or mvolvement of lymphnodes were see”. Despite operatwc intervention and hist&gical diagnosis. liver metas~es (contimxd by needle biopsy) occurred in May 1992. Surambt theraw was beeun at a weeldv dose of 800 m&wxe meter. The drug was stopped in October %92 for i~et7icacy and ;ide effects. The patient presented brain and bond metastases and a serious dermatitis. too. She was hosp”alized +n and Cwhing’s svndrome wilh tv”ical habitus. rmncal oberitv, hinutism. purplish abdominal strine was s&n. Plasml ald&arone conccntmti~n was ., normal value b”t plasma co”isol was 548 microgrunlml and ACTH 4.04 pghnl. The patient was trea!ed with Conone acetate and Aminoelutcmide. a dw? inhibiline glucwa”d mrwralsotico~ds synrhesix Cords01 came back inyo normal valuebut aldost&e inawed. CONCLUSIONS. Primary aldosteronism is an uncommon disease. II results from a” aldosterone-producing adrenal adenoma or. rarely, adrenal carctnoma (less than 1%). Despite operative intervention. most patienu with adnnal carcinoma die within 3 years of diagnosis. Our patient died in April 1994. Suramin is used in the ~atntent of adrenal cancer. ?k number of patients evduated is small and more study is needed to prove its efficacy. We have “or found any report about a switch of hormonal production by adrenal metastatic ctlis aim Suramin therapy. Swamin possesses adrcnoeorticolylic propelries. On human normal adrenocortic~ cells it has a” c”h&itory cffoct on the cy%ochrome P-450 e”zymes. 7he drug might inhibit five adrenal rteroidogenic enzyme3 decreasing the hormone production. It seems that Sum”“” acu on ACTH-receptors. too. To explain the inhibition of aldosterone synthesis and Ihe very high incraw of cotisol plasma level occurred in our patient, we suppose Suranin especially induced a block on a” active, tumoral l&Hydroxylase-enzyme so that tie reaction from DOC to Aldosterone was shut and the accumulation of metabolic middle compounds led to cortisol biosynthesis increase. Other hypotheses could be possible.
CA, Baird
G:,
SlCNIFICANCE OF IN RHEUMATOID Foster H.. Griffiths
Departments of Rheumatology Newcastle upon Tyne
ARTIIRITIS
ID.
and Haematology*.
RVI,
The incidence of Ivm~homa and mveloma are increased in rheumatoid tmhti~is (RA), alth&g’b the reasons kz this arr incompletely nndrrs!wd. We have assessed the predictive value of monoclonal nammo”arhy in RA for the later devcloomeni of lvmohooroliferativc maiienanc’v. A serum paraprorei” was discbvered in 23 p&&s (14 female) wiih .s“&ian age of 59 years and a media” duratlo” of RA of 6 years. The “araurotei” was quantified and funher investigations including a bone m&w &aminatio” and skeletal survey were undenake” to investigate the possible development of a lymphoproliferative disorder. Secondary Sjogren’s Syndrome (SSS) war noted in 12 patients. The pamprotein was moneclonal in 21 patients and was classed as IgG (12 cases). IgA (6 cases) or IgM (3 cases). Biclonal tmraorotcins were see” in 2 w&e”nrs. Free light chains were found in the ;ri”i of 8 patients. Bone%w~ow ab”o&nlttier were found in 13 individuals. I” two thirds of oatients with either an IeA or a” IeM paraprotei” the bane marrow wa; abnormal, whereas less thin half of thise with a” IgG pamprotein had a” abnormal marrow. Patients were followed Up for a media” of 4 years and five patients with a” abnormal bone marrow developed myeloma of whom 3 had a” IgAh paraprotein. Two other patients developed non-Hodgkins lymphoma. Exua-articular bony erosions Were in 4 oatients. three of whom develooed mveloma but the “txsence of urinary f;ee light chains was not sp’ecific’for the dev&pment of malignancy. Paraprotcinsemra I” RA indicacs monoclonal B cell proliferation and carries an increased risk of malignant trasformario”.
AFTER SURAMIN THERAPY, A METASTATIC ADRENAL CARCINOMA SWITCHED FROM PRODUCING ALDOSTERONE TO PRODUCING CORTISOL Ciompi ML’. *Rheumatology
Amaw L*. De Giorgio F.‘. Barn&i L.*. Mmemini R.” Unit and “Department of Surgery-Urology Unit. University
of Piss.
CASE REPORT. Hypertension and hypokalemia appeared in a fifty-years old woman in 19% On treatment with anti-hypertensive drags, she consulted our Rheumatology Unit complaining muscle weakness at her legs and fatigue. Clinical chemistry showed hypokalemia, hypcmaamia, alkaline urine pH, and a primary aldosteronism was supposed. lncmase in plasma aldostemnc coneentratio” (619 pg/ml) and B-scan images ~ontirmd diagnosis. Plasma renin. corurol and A(;TH levela were in normal values. Opratrve tntewe.ntt~n with a&ton of the tumor was ptrformed in Februv 1991. and the hlsmlagical examination showed a macro-adenoma with a “CC~OOCarea inside. No enlargement or mvolvement of lymphnodes were see”. Despite operatwc intervention and hist&gical diagnosis. liver metas~es (contimxd by needle biopsy) occurred in May 1992. Surambt theraw was beeun at a weeldv dose of 800 m&wxe meter. The drug was stopped in October %92 for i~et7icacy and ;ide effects. The patient presented brain and bond metastases and a serious dermatitis. too. She was hosp”alized +n and Cwhing’s svndrome wilh tv”ical habitus. rmncal oberitv, hinutism. purplish abdominal strine was s&n. Plasml ald&arone conccntmti~n was ., normal value b”t plasma co”isol was 548 microgrunlml and ACTH 4.04 pghnl. The patient was trea!ed with Conone acetate and Aminoelutcmide. a dw? inhibiline glucwa”d mrwralsotico~ds synrhesix Cords01 came back inyo normal valuebut aldost&e inawed. CONCLUSIONS. Primary aldosteronism is an uncommon disease. II results from a” aldosterone-producing adrenal adenoma or. rarely, adrenal carctnoma (less than 1%). Despite operative intervention. most patienu with adnnal carcinoma die within 3 years of diagnosis. Our patient died in April 1994. Suramin is used in the ~atntent of adrenal cancer. ?k number of patients evduated is small and more study is needed to prove its efficacy. We have “or found any report about a switch of hormonal production by adrenal metastatic ctlis aim Suramin therapy. Swamin possesses adrcnoeorticolylic propelries. On human normal adrenocortic~ cells it has a” c”h&itory cffoct on the cy%ochrome P-450 e”zymes. 7he drug might inhibit five adrenal rteroidogenic enzyme3 decreasing the hormone production. It seems that Sum”“” acu on ACTH-receptors. too. To explain the inhibition of aldosterone synthesis and Ihe very high incraw of cotisol plasma level occurred in our patient, we suppose Suranin especially induced a block on a” active, tumoral l&Hydroxylase-enzyme so that tie reaction from DOC to Aldosterone was shut and the accumulation of metabolic middle compounds led to cortisol biosynthesis increase. Other hypotheses could be possible.