Original Article
Fas Gene Mutations in the Canale–Smith Syndrome, an Inherited Lymphoproliferative Disorder Associated with Autoimmunity
N Engl J Med 1996; 335:1643-1649November 28, 1996
- Abstract
Background
The Canale–Smith syndrome is a childhood disorder characterized by lymphadenopathy and autoimmunity. The similarity between this syndrome and that in mice with the lymphoproliferation (lpr ) phenotype or the generalized-lymphoproliferative-disease (gld) phenotype led us to investigate whether it too is caused by mutations of the Fas gene (lpr mice) or the Fas ligand (gld mice), which regulate apoptosis in lymphocytes.
Methods
We studied four patients with the syndrome and their families. T-lymphocyte phenotypes were analyzed, and the susceptibility of activated T cells to Fas-mediated apoptosis in vitro was determined. Mutations of Fas were sought by nucleotide-sequence analysis.
Results
Patients with the Canale–Smith syndrome had increased numbers of circulating double-negative T cells (>20 percent) and profoundly impaired apoptosis of activated T cells incubated with an anti-Fas antibody. Three novel Fas mutations were identified, all of which were heterozygous and predicted to impair signal transduction by Fas. Autoimmune manifestations of the disease, such as hemolytic anemia and thrombocytopenia, persisted into adolescence. Two patients followed into adulthood had intermittent lymphadenopathy, which diminished over time. Neoplasms developed in both, and one died of hepatocellular carcinoma at the age of 43.
Conclusions
Patients with the Canale–Smith syndrome have mutations in Fas — a fact that implicates this gene in the accumulation of lymphocytes and the autoimmunity characteristic of the syndrome.
Media in This Article
Figure 2
Fas-Mediated Apoptosis of Activated T Cells in the Patients, Their Relatives, and Normal Controls.Figure 1
Analysis of Lymphocyte Subgroups in Patients with the Canale–Smith Syndrome and Their Relatives, and Control Subjects.Article Activity
- Article
The Canale–Smith syndrome, first described in 1967,1 is an uncommon cause of lymphadenopathy in children.2-4 Patients with the syndrome present within the first two years of life with lymphadenopathy, hepatosplenomegaly, hemolytic anemia, and thrombocytopenia. Lymph-node biopsy reveals nonspecific hyperplasia with increased numbers of lymphocytes, plasma cells, and histiocytes.1 The response to corticosteroids and immunosuppressive drugs varies, and the long-term prognosis is not known; only one patient has been followed into adolescence.
The presence of hypergammaglobulinemia and autoantibodies against erythrocytes and platelets led Canale and Smith1 to postulate that the syndrome had a primary immunologic basis. We studied four patients with the disorder, and all four had mutations in the “death domain” of the Fas receptor (also called APO-1 and CD95). The death domain is the cytoplasmic region of the Fas protein that transduces the intracellular signals required to initiate programmed cell death (apoptosis). Fas is particularly important in the apoptosis of activated lymphocytes5 and macrophages.6 In mice, mutations in the Fas receptor (lpr, lymphoproliferation phenotype) or its ligand (gld, generalized-lymphoproliferative-disease phenotype) are associated with massive lymphadenopathy and lupus-like autoimmunity.5 These animals also have large numbers of T cells with down-regulated CD4 and CD8 surface molecules (“double-negative” T cells). Normally, the Fas pathway triggers apoptosis in the cells. We found increased numbers of these unusual T cells in patients with the Canale–Smith syndrome.
Case Reports
Patient 1
Patient 1 is a 43-year-old woman whose presentation and early history have been described previously.1 At the age of 15, a left suborbital mass developed, which consisted of chronic inflammatory tissue with numerous foreign-body-type multinucleated giant cells. Two years later, thrombocytopenia resulted in severe menometrorrhagia. At the age of 21, pelvic masses were detected and a lymphangiogram revealed extensive bilateral iliac and para-aortic lymphadenopathy. Ten years later, the patient underwent laparotomy for an enlarging abdominal mass, which was found to be a lymph-node aggregate weighing 234 g and measuring 9.5 by 7 by 5 cm. Immunophenotyping of the cells revealed 87 percent CD3+ T cells (pan-T cells; normal range, 48 to 67 percent), 20 percent CD4+ T cells (normal range, 29 to 48 percent); 15 percent CD8+ T cells (normal range, 15 to 27 percent), and 67 percent HLA-DR+ cells (normal range, 6 to 25 percent). These results are consistent with an excess of activated, double-negative T cells (CD3+, CD4-, CD8-). The patient continues to have mild cervical, axillary, and intraabdominal lymphadenopathy. She also has chronic hepatitis B and hepatitis C infections and persistent hypergammaglobulinemia: IgA, 488 mg per deciliter (upper limit of normal, 382); IgM, 239 mg per deciliter (upper limit of normal, 277); and IgG, 4240 mg per deciliter (upper limit of normal, 1685). She has had multiple neoplastic lesions: a breast adenoma (at the age of 22), three thyroid adenomas (at 15, 32, and 36 years of age), and two basal-cell carcinomas (at 22 and 41 years of age). No autoimmunity or lymphadenopathy has occurred in her parents, her six siblings, or her son.
Patient 2
Patient 2, a man who has been described previously,1 presented at the age of 43 with hepatitis associated with hepatitis C infection. A liver biopsy revealed hepatocellular carcinoma, and he died one month later. He had been treated with corticosteroids and mercaptopurine from the ages of 4 to 12, with substantial, but incomplete, regression of lymphadenopathy. The lymphadenopathy gradually diminished during adolescence and was infrequent and mild during adulthood.
Patient 3
Patient 3 is the eight-year-old son of Patient 2. He presented at seven months with a syndrome identical to that of his father. A lymph-node biopsy revealed atypical T-cell hyperplasia with angioimmunoblastic features; the bone marrow aspirate contained no atypical cells. Ultrasonography demonstrated a mass of lymph nodes in the porta hepatis.3 Worsening thrombocytopenia required splenectomy at the age of two years. Subsequently, autoimmune hemolytic anemia and thrombocytopenia were controlled with methotrexate (2.5 mg per week). At present, the child has massive lymphadenopathy (predominantly cervical) but is otherwise well. Serum immunoglobulin levels are normal except for IgA, which is slightly elevated (267 mg per deciliter; upper limit of normal, 202).
Apart from his father, no family member has a history of lymphoproliferative or autoimmune diseases.
Patient 4
Patient 4, an eight-year-old boy, was found to have splenomegaly at 4 months and hemolytic anemia and neutropenia (absolute neutrophil count, 154 per cubic millimeter [normal, 500 to 8500]) at 10 months of age. A bone-marrow biopsy was normal. Because of generalized lymphadenopathy, a lymph-node biopsy was done. It revealed reactive follicular hyperplasia with 24 percent CD4+ T cells (control value, 38 percent), 11 percent CD8+ T cells (control value, 29 percent), 42 percent CD2+ T cells (control value, 79 percent), and 55 percent CD19+ T cells (control value, 7 percent). At three years of age, hypergammaglobulinemia, a positive direct Coombs' test, anti–smooth-muscle antibodies, and an antinuclear-antibody titer of 1:320 with a nucleolar pattern were detected.
At 3 1/2 years of age, profound thrombocytopenia developed; the patient responded to intravenous immune globulin but required splenectomy. At surgery, massive splenomegaly and mesenteric lymphadenopathy were found. Currently, the boy has generalized lymphadenopathy but is otherwise well; serum immunoglobulin levels are normal. The family history is unremarkable except for a grandmother who had multiple sclerosis and a grandfather who had Guillain–Barré syndrome in 1994. The patient has a healthy dizygotic twin.
Methods
Flow Cytometry
Peripheral-blood mononuclear cells were isolated by density-gradient centrifugation and analyzed by flow cytometry 6,7 (FACScan, Becton Dickinson, San Jose, Calif.) with the following primary antibodies: unconjugated rabbit polyclonal antihuman Fas (N18, Santa Cruz Biotechnology, Santa Cruz, Calif.); biotin-conjugated anti-CD3, anti-CD4, anti-CD8, and anti-CD19 (Caltag, South San Francisco, Calif.); biotinylated anti-CD56 (Southern Biotechnologies, Birmingham, Ala.); and phycoerythrin-conjugated anti-CD4, anti-CD8, and anti-CD25 (Becton Dickinson). The secondary reagents used were streptavidin conjugated to fluorescein isothiocyanate (Jackson ImmunoResearch, West Grove, Pa.) or Tricolor (Caltag); and donkey antirabbit IgG conjugated to fluorescein isothiocyanate or phycoerythrin (Jackson ImmunoResearch). Double-negative T cells were detected by three-color staining with anti-CD3, anti-CD8, and anti-CD4 monoclonal antibodies.
Cell Culture and Analysis of Fas and Fas-Ligand Function
T cells were activated with the anti-CD3 monoclonal antibody OKT3 (ascites fluid, 1:1000 dilution) and interleukin-2 (20 U per milliliter) for seven to eight days before the assays. To examine the function of Fas, the activated T cells were incubated with IgG3 anti–APO-18 (kindly provided by Peter Krammer, German Cancer Research Institute, Heidelberg, Germany) or control monoclonal antibody for 16 hours,7 and cell viability was measured by the Alamar Blue assay.6 The function of Fas ligand was evaluated by a chromium-release assay, with activated T cells (>95 percent CD3+) and target cells consisting of a mouse L1210 B-cell lymphoma cell line, transfected with murine Fas in either the sense or antisense orientations (kindly provided by W. Clarke, UCLA, Los Angeles).9 Effector cells were incubated with targets labeled with chromium-51 for six hours in the presence of anti-CD3, and the extent of specific lysis was calculated.6 The assay was also performed in the presence of magnesium ethyleneglycol-bis-(β-aminoethylether)-N,N,N',N'-tetraacetic acid to block calcium-dependent, perforin-mediated cytotoxicity, as described elsewhere.10
Nucleotide Sequence of Fas and Fas-Ligand DNA and Analysis of Single-Strand Conformation Polymorphisms
Complementary DNA (cDNA) was prepared from anti-CD3–activated T cells,11 and Fas and Fas-ligand (FasL) gene sequences were amplified with the polymerase chain reaction (PCR) by the primers12,13 shown in Table 1Table 1
Oligonucleotide Primers Used in Studies of the Fas and FasL Genes.. For Fas, a 694-bp 5' cDNA fragment, spanning sequences that encode the extracellular and transmembrane domains (primer pair 1 and 14 in Table 1), and a 506-bp 3' cDNA fragment, encoding transmembrane and cytoplasmic domains (primers 11 and 4 in Table 1), were generated. The PCR products were cloned into pGEM-T (Promega, Madison, Wis.), and bidirectional sequencing of inserts was performed with consensus T7 and Sp6 primers. The results were assembled as directed by the Prism Dye Terminator Cycle Sequencing Ready Reaction kit (Perkin Elmer, Foster City, Calif.) and analyzed on an automated sequencer (model 377, ABI, Foster City, Calif.).To verify mutations, genomic DNA was isolated from peripheral-blood leukocytes.14 Up to 200 ng of DNA was used as a template in a 100-μl PCR reaction (denaturation at 95°C for one minute, annealing at 55 to 60°C for one minute, and extension at 72°C for one to two minutes) for 30 cycles. The PCR products were sequenced directly with primers end-labeled with [γ32P]ATP (fmol DNA cycle sequencing system, Promega). In the case of Patient 2, archival material was available from his liver-biopsy sample (kindly provided by Dr. A. Altman, Warren Hospital, Phillipsburg, N.J.), and genomic DNA was isolated as described elsewhere.15
Analysis of single-strand conformation polymorphisms was used to investigate the frequency of defined mutations in 100 unrelated subjects. Primers spanning the 5' end of the death domain (cDNA position, 871 to 1055) were used to amplify genomic DNA, as described above. The results were analyzed as reported elsewhere.16
Serologic Analysis
Serum samples were tested for antinuclear antibody by indirect immunofluorescence with Hep2 cells used as the substrate, as described previously.17 Anticardiolipin18 antibodies, antibodies against double-stranded DNA,17 and IgM rheumatoid factors19 were detected by an enzyme-linked immunosorbent assay.
Results
T-Cell Phenotypes
Phenotypic analysis of T cells from the three living patients revealed that all had higher levels of double-negative (CD3+CD4-CD8-) T cells (>20 percent) than control subjects (<5 percent) (Figure 1AFigure 1
Analysis of Lymphocyte Subgroups in Patients with the Canale–Smith Syndrome and Their Relatives, and Control Subjects.). In patient 1, almost half of all T cells in the blood had this unusual phenotype (Figure 1B). The clinically normal twin sister of Patient 4 had intermediate levels of double-negative T cells (12 percent). Fas was detected on unstimulated T cells from all patients (data not shown).Fas-Mediated Apoptosis
Activated T cells from the patients were almost completely resistant to apoptosis induced by ligating the Fas receptor with an anti-Fas antibody (Figure 2Figure 2
Fas-Mediated Apoptosis of Activated T Cells in the Patients, Their Relatives, and Normal Controls.). Activated T cells from the mother and twin sister of Patient 4 (both of whom were phenotypically normal) were also highly resistant to the anti-Fas antibody (Figure 2).To assess the function of the Fas ligand, which mediates one of the cell-mediated cytolytic pathways, we measured the lysis of Fas-positive target cells by activated T cells. The activity of the patients' T cells in this assay was equivalent to that of T cells from normal subjects at all effector-to-target ratios (data not shown). These findings suggest that the expression and function of the Fas ligand are intact in the Canale–Smith syndrome. Consistent with this conclusion is the finding that FasL coding sequences, which were amplified from the patients' cDNA, cloned, and sequenced (six clones for each insert), contained no variations from the published sequence of FasL. 13
Fas Mutations
Since the phenotype of the patients strongly resembled that associated with Fas mutations in mice,20 Fas coding sequences were amplified from cDNA. The PCR products were cloned, and four or more clones were sequenced in both directions for each insert.
In addition to known Fas gene polymorphisms,21 a single nucleotide change, affecting the death domain (amino acid residues 231 to 298), was demonstrated for each patient in half the sequenced 3' cDNA clones. Patient 1 had an insertion of a T at cDNA position 887, which would predict a frame shift leading to a premature termination at residue 230 (K230•). Patient 3 had a transversion of G to T at position 972, resulting in a nonconservative substitution of tyrosine for aspartic acid at residue 244 (D244Y). Patient 4 had a transversion of C to T at position 942 of the Fas cDNA, resulting in an in-frame premature stop codon and truncation at residue 234 (R 234•).
Mutations identified in cloned DNA were confirmed at the genomic level by PCR amplification of a 1.3-kb fragment with primer pair 23 and 4 and direct cycle sequencing of the death domain by primer 17. All four patients were heterozygous with respect to their Fas mutation (Figure 3AFigure 3
Pedigrees of the Patients with the Canale–Smith Syndrome and the Mutations Identified in the Families., Figure 3B, and Figure 3C).Fas Mutations and Defective Fas-Mediated Apoptosis
The mutated Fas allele in Patient 3 was assumed to be derived from his father, Patient 2.1 A Fas mutation in Patient 2 was detected in DNA that was extracted from an antemortem liver biopsy and amplified by PCR (primers 17 and 47). Sequencing of the cloned PCR product revealed the D244Y mutation (data not shown). No Fas mutations were detected in genomic DNA from the son of Patient 1 or the mother of Patient 3.
Analysis of genomic DNA from relatives of Patient 4 revealed that his mother and sister, but not his father, were heterozygous for the R 234• mutation (Figure 3C). The mutations in this family therefore segregated with the in vitro resistance to Fas-mediated apoptosis but not with the expression of disease.
The likelihood that the Fas mutations we found were causally related to the Canale–Smith syndrome was supported by our failure to detect any of the mutations in 100 unrelated subjects by analysis with PCR and single-strand conformation polymorphisms (not shown).
Discussion
We found that, like lpr and gld mice,22 patients with the Canale–Smith syndrome have increased numbers of double-negative (CD3+CD4-CD8-) T cells in the circulation and lymph nodes and profoundly impaired Fas-mediated apoptosis of activated T cells. It is thought that normally activated T cells down-regulate CD4 and CD8 molecules and are disposed of by Fas-mediated apoptosis. But in mice with a mutant Fas gene, they accumulate in vast numbers. Canale and Smith1 commented that the syndrome of chronic lymphadenopathy, hepatosplenomegaly, and autoimmunity named after them did not have a genetic basis, but, prompted by the family history of Patient 3 and the striking features of the murine lpr phenotype present in these patients, we tested the hypothesis that a mutation of the Fas or FasL gene causes the syndrome. The four patients we studied had novel Fas mutations predicted to cause either truncation (K 230• and R 234•) or a nonconservative amino-acid substitution (D244Y) in a highly conserved region of the Fas death domain.23,24
Fas is a member of the superfamily of tumor necrosis factor and nerve growth factor receptors. Binding to its cognate ligand causes clustering of the Fas receptor,25 which recruits signal-transduction molecules to its intracytoplasmic death domain, thereby initiating programmed cell death.24-26 Fas is expressed on thymocytes and activated T and B cells and is thought to be primarily responsible for the apoptosis of antigen-primed, activated lymphocytes.5 Defective Fas function could therefore cause an accumulation of lymphocytes, including potentially autoreactive cells. These molecular abnormalities can account, at least in part, for the lymphadenopathy and autoimmunity characteristic of the Canale–Smith syndrome.
Resistance to Fas-mediated apoptosis in vitro was found in all patients who were heterozygous for a Fas mutation, suggesting that the mutant alleles act in a dominant negative manner. In lpr cg mice a heterozygous amino-acid substitution at position 225 in the Fas death domain results in lymphadenopathy and autoimmunity,27 and in vitro cotransfection of wild-type and mutant Fas genes impairs Fas-mediated apoptosis.28 However, the genotype–phenotype relation is complex. Three members of the family of Patient 4 had the R 234• mutation, as well as defective Fas-mediated apoptosis, but only the proband had autoimmunity and lymphadenopathy. We conclude that a single mutant Fas allele can affect Fas-mediated apoptosis in vitro, but by itself is insufficient to cause disease; additional factors modulate Fas deficiency, as observed in mice bearing the lpr mutation that have different genetic backgrounds.29,30
Our findings are similar to those in the original reports of Fas dysfunction in humans, which also documented inherited, heterozygous Fas mutations with variable penetrance.28,31,32 Comparison of these five cases of a “human autoimmune lymphoproliferative syndrome”28 and three cases of “human lymphoproliferative syndrome and autoimmunity”32 with the Canale–Smith syndrome suggests that all three syndromes are the same.
The major medical complications in infancy and childhood are autoimmune hemolytic anemia, thrombocytopenia, and infection due to splenectomy or neutropenia. Treatment with corticosteroids, immunosuppressive drugs, or both can reduce the degree of lymphadenopathy and improve the cytopenias. However, most of our patients underwent splenectomy during childhood to alleviate the cytopenias.1 The waxing and waning of the lymph nodes suggests that alternative pathways of lymphocyte apoptosis can compensate for impaired Fas function for extended periods. Exacerbations of lymphadenopathy could have been precipitated by viral infections, since certain DNA viruses can inhibit apoptosis.33 Canale and Smith1 observed that bacterial infections frequently cause a reduction in the size of the lymph nodes; bacterial infections induce the release of cytokines such as tumor necrosis factor α that promote lymphocyte apoptosis through alternative pathways.34,35
Our results show that defective Fas function is compatible with long-term survival; two of our patients have been or were under medical care for 29 years. However, lymphadenopathy, autoimmune thrombocytopenia, and complications of blood transfusion (hepatitis virus infection) have continued into adolescence and adulthood. In two patients (Patient 1 and Patient 2) neoplasms developed in adulthood. These tumors could have been related to cytotoxic drugs or hepatitis virus, but a role for Fas mutations requires consideration. Fas is expressed at multiple sites throughout the body, including skin, liver, and gastrointestinal tract.36 Its role in nonlymphoid tissue is not known, but Fas is functional in hepatocytes37,38 and is up-regulated in hepatitis B and C infection.38,39 The failure of cytotoxic T cells to eliminate hepatitis virus through the Fas pathway could therefore have contributed to the persistence of hepatitis virus in Patients 1 and 2. Furthermore, Fas-knockout mice, in which the Fas gene is disabled, have liver hyperplasia, suggesting a role for Fas in controlling the growth of hepatocytes.40 Cells bearing mutant Fas receptors at other sites may also have a growth advantage resulting from the failure of CD8 or natural killer cells to perform tumor surveillance through the Fas effector pathway.41-43 Preliminary studies suggest that some families with Fas mutations have an increased frequency of lymphomas.44
Twelve cases of lymphadenopathy and autoimmunity associated with Fas mutations have now been reported. The characterization of factors that modulate the clinical outcome of Fas mutations may lead to the identification of important susceptibility genes or environmental agents that participate in other autoimmune and lymphoproliferative disorders.
Supported in part by a Specialized Center of Research grant from the National Institutes of Health (SLE P50-AR42588) and by a grant from the Histiocytosis Association of America. Dr. Vaishnaw is a recipient of the Copeman Travelling Fellowship from the Arthritis and Rheumatism Council, United Kingdom.
We are indebted to Drs. M. Hilgartner (New York Hospital, New York), D. Douglas (Children's Hospital of Philadelphia), A. Altman (Warren Hospital, Phillipsburg, N.J.), Steven Halpern (Overlook Hospital, Summit, N.J.), and R. McMillan (Scripps Clinic, La Jolla, Calif.) for patient information; and to Dr. Michael Lenardo (National Institutes of Health, Bethesda, Md.) and our colleagues at the Hospital for Special Surgery for helpful discussions.
Source Information
From the Division of Rheumatology, Hospital for Special Surgery, Cornell University Medical Center, New York (J.D., A.K.V., J.-L.C., K.B.E.), and the Division of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia (K.E.S.).
Address reprint requests to Dr. Elkon at the Hospital for Special Surgery, 535 E. 70th St., New York, NY 10021.
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