Lymphomas and IgA nephropathy

Chiara Cherubini1, Giovanni Barbera1, Salvatore Di Giulio1, Andrea Onetti Muda2 and Tullio Faraggiana2

1 Nephrology–Dialysis, National Institute for Infectious Diseases "L.Spallanzani", IRCCS, Rome, Italy 2 Department of Sperimental Medicine and Pathology, University "La Sapienza", Rome, Italy

Sir,

Usually glomerulonephritis (GN) is a rare complication of lymphomas [1]. Nevertheless the disease is most commonly observed in Hodgkin lymphomas where the most frequent lesion is a glomerular minimal change nephropathy (MCD). Relatively few reports describe GN in non-Hodgkin lymphoma (NHL) patients [2]. In this second type of lymphoproliferative disorder, both T and B-cell types are included.

The patterns of GN described are: (i) MCD, MPGN, FSNGN, and atypical membranous in B-cell NHL; (ii) FSGS in T-cell NHL; (iii) MCD, IgAGN, fibrillary GN, membranous GN, and immune complex GN in mycosis fungoides.

A membranoprolipherative GN has been described in Burkitts’ lymphoma; MCD and membranous GN in lymphosarcoma [3].

Case.

A 44-year-old man was admitted in October 1996 for severe hypertension, nephrotic syndrome, dyspnoea, hyperpyrexia and anaemia. In his history there was a diagnosis of anaemia and chronic sacroiliac osteomyelitis since December 1995, treated with antibiotics without improvement and a total-body-RMN, performed in July 1996, had revealed a mediastinal solid, homogeneous mass. At admission, laboratory tests showed serum glucose 4.25 mmol/l, BUN 14 mmol/l, creatinine 354 µmol/l, creatinine clearance 21 ml/min, uric acid 660 µmol/l, serum total protein 6.4 g/l, albumin 41.8%, {alpha}1 6.6%, {alpha}2 15.6%, ß 11.6%, {gamma} 24.4%, A/G 0.72. Moderate metabolic acidosis and normal electrolyte equilibrium were found. Liver function was normal. HBsAg, HCVAb and HIVAb were negative. WBC 5.2x103/µl, RBC 2.9x103/µl, haemoglobin 4.65 mmol/l, haematocrit 22.7%, PLT 183x103/µl. Plasma iron was 21 µg/dl, transferrin 171 mg/dl and ferritin 393 ng/ml. Urinalysis showed: proteinuria 4 g/24 h, erythrocyturia >30 HPF, hyaline-granular cylindruria, normal urinary proteins on immunoelectrophoresis. IgG 1881 mg/dl, IgA 344 mg/dl, IgM 402 mg/dl; C3 85 mg/dl, C4 37 mg/dl, ANA/DNA 3.6 (n.v. <= 2); cryoglobulins negative; serum {kappa} light chains, 505 mg/dl; serum {lambda} light chains, 291 mg/dl (normal range findings); c-ANCA and p-ANCA negative.

The discordance between normal WBC count and distribution and the diagnosis of osteomyelitis, the persistence of a normosideremic anaemia and the possibility of underestimating the importance of mediastinal mass, prompted a new total-body RMN. The pre-existing findings were confirmed but marked splenomegaly, no adenopathies and normal sized-structured kidneys, were detected. The RM signal by remnant pelvic bones showed the presence of heterotopical erythropoiesis sites.

A bone marrow examination, by needle sampling of the sternum, resulted in normal haematological findings. Just a week after admission, the impairment of renal function parameters necessitated haemodialysis with a three times a week treatment. Biopsies of mediastinal mass, sacroiliac lesion and kidney were also performed. Percutaneous renal biopsy showed a diffuse extracapillary glomerulonephritis with IgA deposits and no vascular lesions (see Figure 1Go). The mediastinal mass histology revealed a Hodgkin lymphoma of scleronodular type. The sacral bone biopsy confirmed an infiltrative focus with lymphoproliferative disorder.



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Fig. 1. Extensive proliferation in the Bowman's space with a crescentic lesion strikingly surrounding the glomerular tuft (Periodic Acid-Schiff, original magnification 350x).

 
Therapy was started with methylprednisolone pulses (1000 mg a day for 3 days) followed by oral prednisone at 1 mg/kg daily doses and chemotherapy including nitrogen mustards, oncovin, procarbazine, prednisone (MOPP) and adriamycin, bleomycin, vinblastine, dacarbazine (ABVD) alternatively in 8 cycles in total. Dialysis was stopped 15 days after the beginning of therapy because of progressive improvement of kidney function. Six months later renal function was completely recovered.

Comment.

An IgAGN in HL was unexpected, particularly in view of the histological patterns of MPGN. The onset of proteinuria and renal failure appears to be subsequent to the development of lymphoma in this patient. However, the clinical follow-up suggests that a lymphoproliferative disorder had started at least 1 year before the presentation of kidney involvement but it was misdiagnosed. The relationship between the evident nephropathy and related lymphoma seems to be strong. The remission of renal involvement after chemotherapy in this patient supports this observation.

A review of the literature shows an association between IgAGN and cutaneous forms of n-HL (mycosis fungoides) [4] and only two reports describe such a pattern of nephropathy in visceral forms [5]. No reports are actually available about IgAGN association with HL.

What kind of relationship may exist between IgA deposition in kidneys and the development of this lymphoproliferative disorder remains an interesting question.

References

  1. Dabbs DJ, Striker LM, Mignon F, Striker G. Glomerular lesions in lymphomas and leukemias. Am J Med1986; 80: l3–70
  2. Bettencourt Fernando P, Faria MS, Capucho R, Costa E, Guerra L, Faria V. Non-Hodgkin's lymphoma and glomerulonephritis. What kind of relation? Nephrol Dial Transplant1996; 11: 854–856[ISI][Medline]
  3. Harper L, Adu D. Glomerulonephritis and non-Hodgkin lymphoma. Nephrol Dial Transplant1997; 12: 1520–1525[Free Full Text]
  4. Ramirez G, Stinson JB, Zawada ET, Moatamed F. IgA nephritis associated with Mycosis Fungoides. Arch Intern Med1981; 141: 1287–1291[ISI][Medline]
  5. Mak SK, Wong PN, Lo KY, Wong AK. Successfull treatment of IgA nephropathy in association with low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue type. Am J Kidney Dis1998; 31: 713–718[ISI][Medline]




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