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Ann Rheum Dis 2001;60:173–176 173 Ann Rheum Dis: first published as 10.1136/ard.60.2.173c on 1 February 2001. Downloaded from chronic illness and generalised symptoms of under imaging guidance, and microbial cul- systemic infection, such as prolonged fever, ture of the causative organism.57If abdominal LETTERS TO malaise, anorexia, weight loss, and anaemia.23 CT or MRI is unavailable, ultrasonography Pain with flexion and external rotation of may demonstrate the inflammatory mass.8 THE EDITOR the aVected hip is the most common physical Gallium-67 scanning may be useful in the finding.1–3 A tender palpable mass may be diagnosis of psoas abscesses and detection of found in the iliac fossa and inguinal area.23 concomitant infectious foci.9 DiVerential diag- Fifty per cent of patients have abdominal noses of psoas abscess include bacterial infec- tenderness, but guarding and rebound ten- tion of the hip, avascular necrosis of the hip, derness are uncommon.3 Because of the non- irritable hip, necrotising fasciitis of the psoas Primary psoas abscess specific pain location, the diagnosis of psoas muscle, pyelonephritis, pelvic inflammatory Primary psoas abscess is a rare infection with abscess may be delayed or missed. DiVeren- disease, retrocaecal appendicitis, S1 herniated an often vague and non-specific clinical pres- tiation between psoas abscess and hip pathol- disc, avascular necrosis, vertebral or pelvic 34 entation, especially in children. In Asia and ogy can be diYcult; however, prudent physi- osteomyelitis, and epidural abscess. These Africa 99.5% of all psoas abscesses are cal examination of the hip can be useful. With entities should be distinguishable upon the primary, compared with 61% in the United psoas abscesses there usually is no discomfort correlation of history, physical examination, on full flexion of the hip, whereas the States and Canada and 18.7% in Europe.12 laboratory tests, and imaging studies. presence of hip pathology typically elicits Approximately 70% of psoas abscesses occur The cause of primary psoas abscess pain.35 Laboratory studies are non-specific in patients younger than 20 years of age, with remains uncertain. Proposed mechanisms of and typically show leucocytosis, anaemia, a a male preponderance of 3:1.1 Fifty seven per psoas abscess formation include haematog- raised erythrocyte sedimentation rate, and, cent of psoas abscesses occur on the right enous spread from primary infectious foci or usually, normal urine analysis.236 side, 40% on the left side, and 3% bilaterally.3 local trauma with intramuscular haematoma Plain abdominal radiographs occasionally We present the following case and show the formation predisposing to abscess develop- define an outline of the inflammatory mass. 6 magnetic resonance imaging to emphasise ment. In secondary psoas abscess the most Chest radiographs may disclose minimal pleu- the presenting signs, symptoms, and findings commonly associated disorder is Crohn’s dis- ral eVusion or raised hemidiaphragm. An of this unusual infection. ease; other disorders include appendicitis, intravenous pyelogram may show deviation of colonic inflammation or neoplasm, disc A 13 year old white girl was in excellent the kidney and ureter. Barium studies may health until she developed a dull ache in the infections, and a variety of intra-abdominal or disclose bowel loop displacement and associ- retroperitoneal infections.1–4 Primary psoas superior posterior thigh without radiation. She ated gastrointestinal diseases.235However, the denied any direct trauma or excessive strenu- abscesses are caused by a single organism in most accurate diagnostic imaging is computed 87.5% of cases: primarily Staphylococcus ous activity. Over five days she developed pro- tomography scan (CT) or MRI, which typi- gressively severe, dull pain, localised to the aureus (88.4%), streptococci (4.9%), and cally show a low density lesion of the psoas Escherichia coli (2.8%).1 Blood cultures are posterior hip in association with fever to muscle and gas within the muscle itself.57 38.9°C, nausea, vomiting, and diarrhoea. She positive in 41.7%, usually for Staphylococcus There may be rim enhancement of the abscess aureus.1 In the past decade the majority of walked with a limp. Her past medical history wall with contrast medium injection. Defini- was non-contributory; she denied smoking, patients with a primary psoas abscess were tive diagnosis is made by fine needle aspiration intravenous drug users (86%) infected with alcohol, drug use, or sexual activity. The girl 7 was 1.52 m tall and weighed 70 kg. Vital signs the human immunodeficiency virus (57%). were normal; temperature rose to 38.9°C Treatment for primary psoas abscess in- cludes percutaneous drainage combined with within 24 hours of admission. A detailed gen- 10 eral physical examination was normal. Ab- systemic antibiotic administration. Surgical dominal and pelvic examinations were benign drainage is preferred for the patients in whom the psoas abscess is associated with underly- without organomegaly or peritoneal signs. 10 Stool for occult blood was negative. Muscu- ing bowel disease. With appropriate treatment, psoas abscess rarely results in death loskeletal examination was normal, with the 1 exception of the left hip which showed pain on (2.5%). Death from psoas abscess is associ- http://ard.bmj.com/ ated more commonly with inadequate or active and passive motion, particularly abduc- 1 tion and medial rotation. The range of motion delayed drainage, or both. Our patient of the hip was normal; there was no localised responded well to antibiotic treatment and warmth or palpation tenderness. The gait was recovered completely. antalgic for the left leg. T THONGNGARM Laboratory examination showed a white Department of Medicine, Siriraj Hospital, blood cell count of 15.2 × 109/l (77% Mahidol University, Bangkok, Thailand neutrophils/14% lymphocytes/8% monocytes) and on September 28, 2021 by guest. Protected copyright. and platelets were 415 × 109/l. An erythrocyte Division of Rheumatology and Molecular sedimentation rate was 115 mm/1st h (normal Immunology, <20 mm/1st h). Urine analysis disclosed trace Figure 1 Coronal magnetic resonance imaging University of Mississippi Medical Centre, blood and protein; the remainder of the scan of the abdomen showing abnormal signal Jackson, MS, USA laboratory tests were within normal limits. intensity in the inferior pole of the left psoas Blood and cervical cultures were negative. muscle (arrows). Note the proximity of the psoas R W MCMURRAY to the femoral head. Posteroanterior radiographic examination of Rheumatology Section/Medicine Service, the left hip was normal. Bone scan was normal. GV (Sonny) Montgomery VA Hospital Magnetic resonance imaging (MRI) of the and abdomen and pelvis showed grossly abnormal Division of Rheumatology and Molecular signal intensities of the left psoas muscle (figs 1 Immunology, and 2). Although a discrete abscess was identi- University of Mississippi Medical Centre, fied, fine needle aspiration under imaging Jackson, MS, USA guidance yielded no pathological material. Correspondence to: Dr R W McMurray, Division of Vancomycin was started empirically, based on Rheumatology and Molecular Immunology, L525 likely causative organisms. The patient defer- Clinical Sciences Building, University of Mississippi vesced, became ambulatory within one week, Medical Centre, 2500 North State Street, Jackson, and was discharged to complete an outpatient MS 39216, USA antibiotic course. This case demonstrates the manifestations of psoas abscess formation. The classical pre- 1 Ricci MA, Rose FB, Meyer KK. Pyogenic psoas abscess: worldwide variations in etiology. World senting symptoms of psoas abscess are pain, J Surg 1986;10:834–43. limp, fever, and psoas spasm.2–4 Pain is most 2 Gruenwald I, Abrahamson J, Cohen O. Psoas abscess: case report and review of the litera- commonly localised to the ipsilateral hip, but Figure 2 Cross sectional magnetic resonance occasionally radiates to the abdominal wall, ture. J Urol 1992;147:1624–6. imaging of the pelvis showing abnormal signal 3 Bresee JS, Edwards BS, Edwards MS. Psoas back, thigh, inguinal area, flank, knee, and intensity of the psoas closely approximating the abscess in children. Pediatr Infect Dis J calf.13 Patients may also present with a bladder (arrow). 1990;9:201–6.

www.annrheumdis.com 174 Letters Ann Rheum Dis: first published as 10.1136/ard.60.2.173c on 1 February 2001. Downloaded from 4 el Hassani S, Echarrab el-M, Bensabbah R, Attaibi A, Kabiri H, Bourki K, et al.Primary psoas abscess. A review of 16 cases. Rev Rhum Engl Ed 1998;65:555–9. 5 Williams MP. Non-tuberculous psoas abscess. Clin Radiol 1986;37:253–6. 6 Isabel L, MacTaggart P, Graham A, Low B. Pyogenic psoas abscess. Aust N Z J Surg 1991; 61:857–60. 7 Santaella RO, Fishman EK, Lipsett PA. Primary vs secondary iliopsoas abscess. Arch Surg 1995;130:1309–13. 8 Chern CH, Hu SC, Kao WF, Tsai J, Yen D, Lee CH. Psoas abscess: making an early diagnosis in the ED. Am J Emerg Med 1997;15:83–8. 9 Kao PF, Tzen KY, Tsui KH, Tsai MF, Yen TC. The speciﬁc gallium-67 scan uptake pattern in psoas abscesses. Eur J Nucl Med 1998;25: 1442–7. 10 McAuliVe W, Clarke G. The diagnosis and treatment of psoas abscess: a 12 year review. Aust N Z J Surg 1994;64:413–17.

Klippel-Feil syndrome in the prehispanic population Figure 2 (A) Atlas with a hypoplastic left arch; the lack of fusion of the anterior arch of the left of El Hierro (Canary foramen transversarium is also evident. (B) Left hemiatlas. Islands) with sharp, fine proximal, and distal ends clinical expression of the Klippel-Feil syn- Klippel-Feil syndrome is an uncommon (fig 2A), supporting an underlying devel- drome is variable. alteration (1:40 000 births), mainly aVecting opmental defect rather than an acquired the cervical spine.1 The classic clinical triad one. E GONZÁLEZ-REIMERS A MAS-PASCUAL described by Klippel and Feil in 1912—short 4 A left hemiatlas (fig 2B); although it is pos- Dpto de Medicina Interna, neck, low dorsal hairline, and restricted neck sible that the right body of the bone was Hospital Universitario de Canarias, mobility—is the result of the fusion of a vari- partially fused with the left one (and was Tenerife, Canary Islands able number of cervical vertebrae, sometimes not recovered in the archaeological excava- reducing their number, and cervical spina tion), the posterior end of the arch was M ARNAY-DE-LA-ROSA bifida.2 Extraosseus changes,3 hemivertebra, neither fractured nor fused to any other J VELASCO-VÁZQUEZ vertebral body clefts, and thoracolumbar bony structure, thus pointing to a develop- M C JIMÉNEZ-GÓMEZ abnormalities,4 are sometimes seen. mental defect. Dpto de Prehistoria, Antropología e Historia In a prehispanic ossuary containing re- Fusion of C2 and C3 (and C5-6), hypopla- Antigua, mains of at least 121 subjects in the island of sia of the arch of the atlas,5 and complete Universidad de la Laguna, El Hierro (Canary Islands), we noted: bipartition of the atlas6 constitute distinctive Tenerife, Canary Islands 1 A C2-3 block, with the two vertebrae fused features of Klippel-Feil disease. Thus the Correspondence to: Dr González-Reimers both by the vertebral bodies and the medial subject with the fused C2-3 and C5-6 blocks ends of the archs, well preserved right and the hemiatlas was probably aVected by intervertebral foramina, and foramina this disease. Possibly, the second atlas belongs 1 Thomsen MN, Schneider U, Weber M, Johain- transversaria (fig 1), and a normal med- nisson R, Niethard FU. Scoliosis and congeni- to another subject with the same disease, tal anomalies associated with Klippel-Feil syn- ullar canal. The body of the third vertebra though this possibility should be cautiously drome types I-III. Spine 1997;22:396–401. shows intense degenerative signs. admitted. The two atlas bones show diVerent 2 Chaumien JP, Rigault P, Maroteaux P, Padovani http://ard.bmj.com/ 2 A C5-6 block, consisting of two vertebrae JP, Touzet T. Le soi-disant syndrome de developmental abnormalities. In the new- Klippel-Feil et ses incidences orthopediques. with intense degenerative changes fused born, the ossification of the cartilaginous Rev Chir Orthop 1990;76:30–8. both by the vertebral bodies and the medial anterior and posterior archs of the atlas takes 3 Hensinger RN, Lang JE, McEwen GD. Klippel part of the archs. Both cervical blocks seem place progressively from the already ossified Feil syndrome. J Bone Joint Surg Am 1974;56: to belong to the same subject. 1246–53. lateral masses. Often, especially in the 4 David KM, Copp AJ, Stevens JM, Hayward RD, 3 A well preserved atlas bone, with an anterior arch, secondary ossification centre(s) Crockard HA. Split cervical spinal cord with incomplete anterior part of the left arch, appear.7 In our case it seems that hypoplastic Klippel-Feil syndrome: seven cases. Brain development of the anterior left arch took 1996;119:1859–72. 5 Chigira M, Kaneko K, Mashio K, Watanabe H. on September 28, 2021 by guest. Protected copyright. place. Because the hypoplastic part of the Congenital hypoplasia of the arch of the atlas arch is in its middle part, probably, a second with abnormal segmentation of the cervical ossification centre was present, but ossifica- spine. Arch Orthop Trauma Surg 1994;113: 110–12. tion was never completed; in this sense, it is 6 Wolf RF, Klein JP. Complete bipartition of the similar to the case described by Chigira et al,5 atlas in the Klippel-Feil syndrome. A radiologi- which also showed fusion of C5-6. cally illustrated case report. Surg Radiol Anat The “hemiatlas” perhaps is really an atlas 1997;19:339–40. 7 Williams PL, Warwick R, eds. Gray’s anatomy. with a midline cleft and a lost half, though the Philadelphia: Saunders, 1980. posterior arch does not reach the midline, so 8 Clarke RA, Catalan G, Diwan AD, Kearsley JH. it never became fused with the right half of Heterogeneity in Klippel-Feil syndrome: a new classification. Pediatr Radiol 1998;28:967–74. the bone. A secondary posterior ossification centre sometimes appears during the first years of life. In this case, it was absent, in contrast with the anterior secondary ossifica- Haematopoietic stem cell tion centre which was surely present in the transplantation (HSCT) in former case. Perhaps familial links existed between the a patient with Sjögren’s two subjects. Klippel- Feil syndrome is a syndrome and lung malt heterogeneous disorder, showing diVerent lymphoma cured alterations in diVerent families.8 The simulta- neous finding of diVerent developmental lymphoma not the abnormalities of the atlas in our two cases— autoimmune disease assuming that the second one truly represents a case of Klippel-Feil—may either reflect an Haematopoietic stem cell transplantation unusually high prevalence of this entity in the (HSCT) has been used in an attempt to con- prehispanic population of El Hierro, or may trol autoimmune diseases that respond Figure 1 Fused C2-3 vertebral block. also indicate that even in the same family poorly to conventional treatment, or as a way

www.annrheumdis.com Letters 175 Ann Rheum Dis: first published as 10.1136/ard.60.2.173c on 1 February 2001. Downloaded from of readjusting the immunological balance.1 Table 2 Report of the European group4 on the three major rheumatological autoimmune diseases As far as we know, only one case of primary treated with autologous stem cell transplantation Sjögren’s (SS) has been reported,2 with an unfavourable outcome. Another patient re- Transplant related death ceived an allogeneic bone marrow transplant Autoimmune disease Number Died (No (%)) (No (%)) 3 and also had an unfavourable outcome. We Systemic sclerosis 33 13 (39) 9 (27) describe here a further patient with primary SLE 14 2 (14) 2 (14) SS who underwent HSCT for a non- RA 35 1 (3) 1 (3) Hodgkin’s lymphoma aVecting the lung Sjögren’s syndrome (present series) 2 1 (50) 0 (large cell, mucosa associated lymphoid Total 84 17 (20) 12 (14) tissue (MALT) lymphoma) and review the literature on the eVects of HSCT on the 6 autoimmune features and histopathological In table 1 we give the characteristics of the intrinsic stem cell defect, does not represent changes in primary SS. other patient with primary SS (No 2), previ- a definite cure either and the related ously reported. It can be seen that the condi- morbidity-mortality still remains too high to tioning regimen, myeloablation, previous be accepted as a possible alternative. As ben- Case report treatment, stem cell rescue, and bone marrow efits have been seen in around two thirds of A white woman, aged 42, developed recur- reconstitution were diVerent. However, in the cases treated so far, controlled trials in the rent parotid swelling and symptomatic sicca this case, also, SS was not cured and there three major rheumatic diseases are eagerly syndrome, with a Schirmer’s test I of 5 mm in was no remission. An immunological reas- waited. the right eye and 4 mm in the left eye. Break sessment showed persistence of the immuno- We gratefully acknowledge the invaluable up time was 6 s and sialometry was <1 ml. logical imbalance and poor function of the help we received from Professor A Tyndall, She had periodic relapses of her parotid salivary apparatus. who provided us with the latest data available swelling. In August 1994 (aged 57) lung x Table 2 shows the results for patients with of the EBMT/EULAR registry on haemato- rays and computed tomography disclosed a three more common autoimmune rheumatic poietic stem cell transplantation in autoim- parenchymal nodule of 3 cm in diameter in diseases (rheumatoid arthritis (RA), systemic mune diseases. the basal left lobe. She underwent a lobec- lupus erythematosus (SLE), and scleroderma tomy that disclosed a MALT, of the large cell (SSc)) treated with HSCT and who received G FERRACCIOLI R DAMATO B lymphoma histotype, stage IE. In Decem- an adequate follow up.4 A total of 270 such ber 1994 two more nodules in the right lobe, SDEVITA patients are registered so far in the European Division of Rheumatology, with hilar bilateral adenomegaly, led to the Bone Marrow Transplant/EULAR database, diagnosis of a relapse of her lymphoma, Udine Medical School, but the number who have received adequate University of Udine, which had progressed to stage IV. She then follow up is much smaller. 33100 Udine, Italy received six courses of F-MACHOP (vincris- Current data suggest that best results have tine 0.5 mg/m2 at hours 0 and 12; cyclophos- been obtained in RA, the worst in SSc, R FANIN phamide 800 mg/ m2 intravenous (IV) bolus suggesting that T helper 2 oriented diseases D DAMIANI at hour 36, 5-fluorouracil 15 mg/kg IV for six have a poorer response. M BACCARANI hours at hour 36, cytosine-arabinoside 1000 Results for SS seem to confirm this Division of Haematology, mg/m2 IV for six hours at hour 42, doxoru- because HSCT cured lymphoma but did not Udine Medical School 2 bicin 60 mg/m IV bolus at hour 48, improve the autoimmune disease. No [email protected] 2 methotrexate 500 mg/m IV for six hours at changes were recorded in the function of sali- [email protected] hour 60, prednisone 60 mg/m2 from day 1 to vary glands, or in the synthesis of ANA, or the 14), and folinic rescue (20 mg/m2, IV bolus at histopathology. The other case reported did 1 Marmont AM. New horizons in the treatment of hours 84, 96, 108, 120), with a prompt show some early improvement in the function autoimmune diseases: immunoablation and reduction of hilar adenopathies and a net of the glands, but no improvement afterwards stem cell transplantation. Annual Reviews in decrease of pulmonary nodule size. However, and an infection leading to death. Early Medicine 2000;51:115–34. 2 Rosler W, Manger B, Repp R, Kalden J, Gram- http://ard.bmj.com/ no complete remission was recorded. She was recurrence of autoimmune features and of atski M. Autologous PBPCT in a patient with then oVered the possible chance of an HSCT. autoantibodies was seen in patients with SLE lymphoma and Sjögren’s syndrome: complete Table 1 (patient 1) reports the myeloablation, and CREST.5 We do not know whether vari- remission of lymphoma without control of the conditioning, recovery, and reinfusion of ous conditioning regimens or myeloablation autoimmune disease. Bone Marrow Transplant 1998;22:211–13. stem cells. After three years of follow up no approaches (with or without T cell depletion) 3 Minowa R, Miyagawa S, Fukumoto T, Majima relapse of the lymphoma has occurred. Sicca might result in diVerent outcomes. It seems T, Shimoyama T, Fujimura Y, et al.Primary syndrome after transplantation was unmodi- unlikely that T cell depletion would oVer a Sjögren’s syndrome followed by chronic myel- ogenous leukemia: a case report with a ten year fied, however, with a persistently poor better prospect, especially in view of the history. J Dermatol 1998;25:460–4. function of the salivary glands, an unchanged increased risk of long term immunosuppres- 4 Tyndall A, Gratwohl A. Immune ablation and on September 28, 2021 by guest. Protected copyright. serology (antinuclear antibody titre 1/2560), sion, lymphoproliferative diseases, and infec- stem-cell therapy in autoimmune diseases. and an unchanged histopathology tions. On the other hand, allogeneic bone Clinical experience. Arthritis Research (in press). (Chisholm-Mason grading = 4) despite marrow transplantation, even though clearly 5 Euler HH, Marmont AM, Bacigalupo A, having mild fibrosis of the salivary glands. appealing given the chance of eradicating the Fastenrath S, Dreger P, HoVnecht M, et al.

Table 1 Lymphoma characteristics, conditioning regimens, side eVects and outcome of the two female patients so far studied, after haematopoietic stem cell transplantation

Variables Patient 1 Patient 2 Age (years) 57 34 Diagnosis NHL* of the lung (large cells MALT* lymphoma) Immunoblastic B lymphoma First line treatment 6 cycles of F-MACHOP* VACOP-B* followed by VIPE* Conditioning regimens ARA-C* 200 mg × 2/day × 4 BCNU 300 mg/m2 VP16* 200 mg × 2/day × 4 Etoposide 800 mg/m2 CTX* 1500 mg/day × 4 Cytarabine 1600 mg/m2 BCNU* 200 mg Melphalan 140 mg/m2 Reinfusion MNC* 4.5 × 108/kg MNC 2.42 × 108/kg CD34 0.9 × 106/kg CD34 4.60 × 106/kg CD3 not counted CD3 37.03 × 106/kg Days to PMN* >1 × 109/l=10days DaystoPMN>10×109/l=11days Days to Plt* >20 × 103/l=8days DaystoPlt>50×103/l=18days Sides eVects/outcomes Alive in complete continous lymphoma remission after 3 years No remission of autoimmune disease No remission of autoimmune disease Died 20 months after transplantation for Pneumocystis carinii pneumonia

*NHL = non-Hodgkin’s lymphoma; MALT = mucosa associated lymphoid tissue; F-MACHOP: F = 5-ﬂuorouracil, M = methotrexate, A = Adriamycin, C= cyclophosphamide, H = doxorubicin, O = Oncovin, P = prednisone; ARA-C = arabinoside-C; VP16 = etoposide; CTX = cyclophosphamide; BCNU = carmustine; MNC = mononuclear cells; PMN = polymorphonuclear cells; Plt = platelets; VACOP-B: V = Vepesid, A = Adriamycin, C = cyclophosphamide, O = vincristine, P = prednisone, B = bleomycin; VIPE: V = vinblastine, I = ifosfamide, P = prednisone, E = etoposide.

www.annrheumdis.com 176 Letters Ann Rheum Dis: first published as 10.1136/ard.60.2.173c on 1 February 2001. Downloaded from Early recurrence or persistence of autoimmune Behçet’s disease, amyloidosis, and with vari- diseases after unmanipulated autologous stem ous neoplastic diseases,4–7 but its onset during cell transplantation. Blood 1996;88:3621–5. 6 Ikehara S. Bone marrow transplantation for the course of polymyalgia rheumatica has not autoimmune diseases. Acta Haematol 1998;99: been recently reported. No data exist about 116–32. the possible relation between Castleman’s disease and polymyalgia, but a role for interleukin 6 can be suggested,8–10 perhaps through Castleman’s disease a stimulating action of this cytokine on monocytes and lymphocytes. The present A 65 year old woman presented in February case can be considered typical of the solitary 1998 with joint pains, mild weight loss, and a form, hyaline-vascular type of Castleman’s low grade irregular fever. Initially, pain was disease. Our patient did not present any other localised around both shoulder joints. Subse- lymphatic disease during the subsequent quently, elbows, wrists, hips, and knees were months, which would seem to confirm the aVected, with morning stiVness, but without good prognosis of this disease. particular swelling. The symptoms were accentuated by movement, but also persisted A RICCIO D NATALE during the night, often keeping the patient Figure 1 The post-capillary vessels penetrate awake. On clinical examination, there was the expanded follicles perpendicularly (“lollipop Dipartimento di Medicina Clinica e limited painful movement of the shoulders image”). (×400.) Sperimentale and hips, with a marked reduction in G PETTINATO strength. The small joints of the hands and centres were depleted of lymphocytes, and L SPARANO feet were not aVected. No other pathological consisted predominantly of dendritic reticu- Dipartimento di Scienze Biomorfologiche e conditions were found. Laboratory findings lar cells showing vascular proliferation. The Funzionali, showed a marked increase in erythrocyte mantle zone was expanded and concentri- Sezione di Anatomia ed Istologia Patologica sedimentation rate (ESR; >100 mm/1st h), cally arranged around the atrophic germinal A PETITO hyper-á globulinaemia and a mild anaemia, 2 centres (onion skin layers). The interfollicular A PUZZIELLO whereas enzymatic activity (serum aspartate areas were also prominent, containing small Dipartimento di Chirurgia Generale Geriatria aminotransferase, serum alanine aminotrans- lymphocytes, occasional eosinophils, plasma Oncologica e Tecnologie Avanzate ferase, alkaline phosphatase, lactate dehydro- cells, and some immunoblasts, and showing Federico II University Medical School, genase, and creatine kinase) was within the numerous hyperplastic vessels of the post- Napoli, Italy normal range. A diagnosis of polymyalgia capillary venous type. Occasionally these ves- rheumatica was made and a rapid and sels, which were often hyalinised, penetrated Correspondence to: Dr A Riccio, via Aniello marked clinical improvement was obtained the expanded follicles perpendicularly, giving Falcone, 388—80127, Napoli, Italy with low dose steroid treatment (prednisone rise to the so-called “lollipop” appearance (fig 12.5 mg/day). Pain disappeared, muscle 1 Isaacson PG. Commentary: Castleman’s dis- 1). An immunocytochemical study con- ease. Histopathology 1989;14:429–32. strength and joint function became normal firmed the normal organisation of the nodal 2 Keller AR, Hochholzer L, Castleman B. within a week. A decrease in ESR (to 40 structures, with a clear positivity of follicular Hyaline-vascular and plasma-cell types of giant mm/1st h) and á globulinaemia was noted lymph node hyperplasia of mediastinum and 2 elements for typical B cell markers CD20 and other locations. Cancer 1972;29:670–83. after one month. The clinical condition CD79a, and positivity of interfollicular lym- 3 Hsu SM, Waldron JA, Xie SS, Barlogie B. remained satisfactory during 1998, with a phoid elements for T cell markers CD3 and Expression of interleukin 6 in Castleman’s disease. Hum Pathol 1993;24:833–9. complete normalisation of ESR and á2 CD45R0, whereas the dendritic reticular 4 Frizzera G. Castleman’s disease and related dis- globulinaemia after three months. cells showed a typical positivity for CD21. orders. Semin Diagn Pathol 1988;5:346–64. In January 1999 she developed jaundice These features are distinctive of Castle- 5 Ordi J, Grau JM, Junque A, Nomdedeu B, Pal- with pruritus and a subcontinuous fever man’s disease, also known by the descriptive acin A, Cardesa A. Secondary (AA) amyloidosis associated with Castleman’s disease. Report (38.5–39°C). Axial tomography and nuclear http://ard.bmj.com/ term angiofollicular lymph node hyperplasia. of two cases and review of the literature. Am J magnetic resonance disclosed compression of This is a clinical entity characterised by Clin Pathol 1993;100:394–7. the biliary duct by a compact, well defined angiofollicular hyperplasia of the lymph 6 Vasef M, Katzin WE, Mendelsohn G, Reydman retropancreatic mass (about 4 cm in diam- nodes without the presence of any atypical M. Report of a case of localized Castleman’s disease with progression to malignant lym- eter). The laboratory pattern was typical of cells or other signs of malignancy. Many phoma. Am J Clin Pathol 1992;98:633–6. cholestasis (hyperbilirubinaemia, increased (multicentric or systemic form) or single 7 Gould SJ, Diss T, Isaacson PG. Multicentric serum alkaline phosphatase activity). Endo- (monocentric or solitary form) lymph nodal Castleman’s disease in association with a 1 solitary plasmacytoma. A case report. Histopa- scopic retrograde colangiopancreatography groups can be aVected in the process and two thology 1990;17:135–40. showed slight enlargement of the biliary tract, histological subsets have been recognised: a 8 Dasgupta B, Panayi GS. Interleukin-6 in serum dilatation of the gall bladder walls, and hyaline-vascular type, characterised by of patients with polymyalgia rheumatica and on September 28, 2021 by guest. Protected copyright. absence of gall stones. Transit of contrast marked expansion of the mantle follicular giant cell arteritis. Br J Rheumatol 1990;29: 456. medium along the biliary tract slowed down. zone and a plasma cell type, with diVuse 9 Higashi K, Matsuki Y, Hidaka T, Aida S, Suzuki Explorative laparotomy confirmed the plasma cell proliferation in the interfollicular K, Nakamura H. Primary Sjögren’s syndrome presence of a mass compressing the common tissue.2 The cause of the disease is unknown, associated with hyaline-vascular type of Castle- 3 man’s disease and autoimmune idiopathic bile duct. This was removed and histological but overproduction of interleukin 6 has been thrombocytopenia. Scand J Rheumatol 1997; examination disclosed a lymph nodal archi- shown in the course of Castleman’s disease 26:482–4. tecture characterised by evident follicular and a possible pathogenic role for this 10 Lee M, Hirokawa M, Matuoka S, Hasegawa H, hyperplasia. Some of the germinal centres cytokine has been suggested. It is commonly Nanjo H, Matsushima T, et al. Multicentric Castleman’s disease with an increase serum were enlarged and comprised polymorphous associated with several autoimmune condi- level of macrophage colony stimulating factor. follicular centre cells, whereas other germinal tions such as systemic lupus erythematosus, Am J Hematol 1997;54:321–3.