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Autoimmune Haemolytic Anaemia Associated with Rheumatoid Arthritis and Paroxysmal Nocturnal Haemoglobinuria

Autoimmune Haemolytic Anaemia Associated with Rheumatoid Arthritis and Paroxysmal Nocturnal... Correspondence © 1986 S. Karger AG, Basel 0001-5792/86/0754-0241 $2.75/0 A ctahaem at. 75: 241 (1986) Autoimmune Haemolytic Anaemia Associated with Rheumatoid Arthritis and Paroxysmal Nocturnal Haemoglobinuria D. Maharaj Royal Infirmary, Glasgow, UK Forman et al. [1984] have described 2 patients with Immunohaematological studies revealed strongly paroxysmal nocturnal haemoglobinuria (PNH) and positive direct Coombs’ test with broad-spectrum an­ autoimmune haemolytic anaemia. More recently, tiglobulin serum. Further studies with monospecific Conti et al. [1985] described a patient with PNH with antiglobulin serum revealed an IgG- and C3d-coating autoimmune haemolytic anaemia and autoimmune on the patient’s cells. The eluate prepared from the thrombocytopenia. I would like to report a patient patient’s erythrocytes had anti-c rhesus specificity. with PNH and autoimmune haemolytic anaemia as­ Ham’s test, sucrose lysis test and insulin test were all positive. sociated with rheumatoid arthritis. An 82-year-old lady originally presented in June The patient has refused treatment with steroids be­ 1976 with aplastic anaemia confirmed by bone mar­ cause of previous side-effects, she is therefore receiv­ row biopsy. She was treated with regular red cell ing regular red cell transfusions of washed cells. In transfusions and oxymetholone 150 m g/day, and a addition, she is taking oral iron and folic acid. Evi­ partial remission was achieved after 6 months. Oxy­ dence of continuing mild haemolysis persists. metholone had to be stopped because of the unaccept­ This is another case of a rare association between able side-effect o f hirsuitism, but in spite of this, par­ PNH and autoimmune haemolytic anaemia o f which tial remission was maintained. In May 1980, the lady only 3 cases have so far been described. Further stud­ developed seropositive rheumatoid arthritis affecting ies are required to determine the incidence of autoim­ mainly the hands and knees. She subsequently re­ mune disorders in PNH patients. quired regular red cell transfusions for an unex­ plained iron deficiency anaemia. In July 1985, she developed haemoglobinuria and References was admitted for further investigation. Examination Forman, K.; Sokol, R.J.; Hewitt, S.; Stamps, B.K.: Paroxysmal noc­ revealed pallor, mild jaundice and typical rheumatoid turnal haemoglobinuria. A clinicopathological study o f 26 joint deformities of her hands and knees. The spleen cases. Acta haemat. 71: 217-226(1984). was moderately enlarged. Conti, L.; Iurlo, A.; G andolfo, G.M.; Girelli, G.: Evans syndrome Investigations: Hb 6.5 g/dl, WBC 3 .8 x l 0 9/ l, in paroxysmal nocturnal haemoglobinuria. Acta haemat. 73: platelets 100 x 109/1, reticulocyte count 14%. Periph­ 210-211 (1985). eral blood film showed spherocytosis and poly- chromasia. Bone marrow biopsy showed reduced cellularity with absent iron stores. Biochemistry: total bilirubin 50 ¿rmol/1, direct bilirubin 9 /imol/1, AST Accepted: May 13,1986 34 U /l, ALT 16 U /l, LDH 2, 160 U /l. Immunoglobu­ lins were normal. Plasma haemoglobin was elevated Dr. D. M aharaj, at 99 mg/1 with absent serum haptoglobin, and uri­ Senior Registrar in Haematology, nary haemosiderin was positive. Rheumatoid and an­ Royal Infirmary, Glasgow G4 0SF (UK) tinuclear factors were positive. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Acta Haematologica Karger

Autoimmune Haemolytic Anaemia Associated with Rheumatoid Arthritis and Paroxysmal Nocturnal Haemoglobinuria

Acta Haematologica , Volume 75 (4): 1 – Jan 1, 1986

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Publisher
Karger
Copyright
© 1986 S. Karger AG, Basel
ISSN
0001-5792
eISSN
1421-9662
DOI
10.1159/000206134
Publisher site
See Article on Publisher Site

Abstract

Correspondence © 1986 S. Karger AG, Basel 0001-5792/86/0754-0241 $2.75/0 A ctahaem at. 75: 241 (1986) Autoimmune Haemolytic Anaemia Associated with Rheumatoid Arthritis and Paroxysmal Nocturnal Haemoglobinuria D. Maharaj Royal Infirmary, Glasgow, UK Forman et al. [1984] have described 2 patients with Immunohaematological studies revealed strongly paroxysmal nocturnal haemoglobinuria (PNH) and positive direct Coombs’ test with broad-spectrum an­ autoimmune haemolytic anaemia. More recently, tiglobulin serum. Further studies with monospecific Conti et al. [1985] described a patient with PNH with antiglobulin serum revealed an IgG- and C3d-coating autoimmune haemolytic anaemia and autoimmune on the patient’s cells. The eluate prepared from the thrombocytopenia. I would like to report a patient patient’s erythrocytes had anti-c rhesus specificity. with PNH and autoimmune haemolytic anaemia as­ Ham’s test, sucrose lysis test and insulin test were all positive. sociated with rheumatoid arthritis. An 82-year-old lady originally presented in June The patient has refused treatment with steroids be­ 1976 with aplastic anaemia confirmed by bone mar­ cause of previous side-effects, she is therefore receiv­ row biopsy. She was treated with regular red cell ing regular red cell transfusions of washed cells. In transfusions and oxymetholone 150 m g/day, and a addition, she is taking oral iron and folic acid. Evi­ partial remission was achieved after 6 months. Oxy­ dence of continuing mild haemolysis persists. metholone had to be stopped because of the unaccept­ This is another case of a rare association between able side-effect o f hirsuitism, but in spite of this, par­ PNH and autoimmune haemolytic anaemia o f which tial remission was maintained. In May 1980, the lady only 3 cases have so far been described. Further stud­ developed seropositive rheumatoid arthritis affecting ies are required to determine the incidence of autoim­ mainly the hands and knees. She subsequently re­ mune disorders in PNH patients. quired regular red cell transfusions for an unex­ plained iron deficiency anaemia. In July 1985, she developed haemoglobinuria and References was admitted for further investigation. Examination Forman, K.; Sokol, R.J.; Hewitt, S.; Stamps, B.K.: Paroxysmal noc­ revealed pallor, mild jaundice and typical rheumatoid turnal haemoglobinuria. A clinicopathological study o f 26 joint deformities of her hands and knees. The spleen cases. Acta haemat. 71: 217-226(1984). was moderately enlarged. Conti, L.; Iurlo, A.; G andolfo, G.M.; Girelli, G.: Evans syndrome Investigations: Hb 6.5 g/dl, WBC 3 .8 x l 0 9/ l, in paroxysmal nocturnal haemoglobinuria. Acta haemat. 73: platelets 100 x 109/1, reticulocyte count 14%. Periph­ 210-211 (1985). eral blood film showed spherocytosis and poly- chromasia. Bone marrow biopsy showed reduced cellularity with absent iron stores. Biochemistry: total bilirubin 50 ¿rmol/1, direct bilirubin 9 /imol/1, AST Accepted: May 13,1986 34 U /l, ALT 16 U /l, LDH 2, 160 U /l. Immunoglobu­ lins were normal. Plasma haemoglobin was elevated Dr. D. M aharaj, at 99 mg/1 with absent serum haptoglobin, and uri­ Senior Registrar in Haematology, nary haemosiderin was positive. Rheumatoid and an­ Royal Infirmary, Glasgow G4 0SF (UK) tinuclear factors were positive.

Journal

Acta HaematologicaKarger

Published: Jan 1, 1986

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