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There are a few reports on celiac associated leukemia. I have appended five citations, two of which are accompanied by abstracts. Although the association does not appear to be common, it is difficult to tell if that is due to the low level of suspicion of celiac disease in general, or if it is due to a rare association. The findings of Fundia et al. (6) suggest that the association may be more common. Please not that Molitor et al. (1) talk about an " intriguing association of celiac disease with T-LGL leukemia." It may also be that the report by Chapman et al.(2) may reflect both the Fundia et al.(6) findings, as well as suggesting an indirect connection between pernicious anemia, which is often found in celiac disease and the high incidence of leukemia in pernicious anemia (7).

  1. Molitor JL, Saint-Louis J, Louvet C, Vachon A, Vincent L, Beaulieu R [Large granular T-cell lymphocytic leukemia disclosed by bilateral uveitis: association with celiac disease]. Rev Med Interne 1997;18(3):237-9
    A 70-year-old woman presented with bilateral anterior uveitis. She was on a gluten-free diet because of a celiac disease which had been diagnosed 3 months before. An anterior chamber aspirate contained a majority of large granular lymphocytes (LGL). The investigation of a chronic neutropenia led to the diagnosis of an otherwise typical T-LGL leukemia. This seems to be the first report of a CD3+ CD4- CD8+ T-LGL leukemia causing anterior uveitis through infiltration of leukemic cells, and the second report of an intriguing association of celiac disease with T-LGL leukemia.

  2. Chapman CS, Mitchell VE, Alexander CP, Potter AM Loss of Philadelphia chromosome in chronic myeloid leukaemia associated with coeliac disease and splenic atrophy. Br Med J (Clin Res Ed) 1988 Jun 4;296(6636):1574-5

  3. Trejdosiewicz LK, Malizia G, Oakes J, Losowsky MS, Janossy G Expression of the common acute lymphoblastic leukaemia antigen (CALLA gp100) in the brush border of normal jejunum and jejunum of patients with coeliac disease.J Clin Pathol 1985 Sep;38(9):1002-6
    Expression of the gp100 common acute lymphoblastic leukaemia antigen (CALLA) was studied in the mucosa of the gut by means of indirect immunofluorescence on cryostat tissue sections with a panel of eight monoclonal antibodies to common acute lymphoblastic leukaemia antigen (anti-CALLA antibodies) and two antibodies to non-CALLA leukaemic antigens. Expression of CALLA was absent from normal stomach epithelium, adult and fetal colonic epithelium of normal histology, and colonic epithelium from patients with Crohn's disease or ulcerative colitis. By contrast, all eight anti-CALLA antibodies gave a characteristic reaction in normal adult and fetal small bowel mucosa, with specific localisation to the entire brush border of jejunal epithelium. Whereas seven of these antibodies reacted both with normal jejunal epithelium and with the damaged epithelium of patients with coeliac disease, antibody RFAL-2 reacted strongly only with histologically normal small bowel but more weakly in patients with coeliac disease to a degree related to the amount of histological abnormality. Expression of the moeity like CALLA identified with RFAL-2 was strongest in crypt epithelium and proportionally diminished along the villi according to the amount of histological damage in coeliac disease, being essentially absent in patients with "subtotal villous atrophy."

  4. Re G, Zaccaria A, Calabrese L, Gobbi M, Lauria F, Cavalli G Hairy-cell leukemia complicating coeliac disease: report of a case and discussion of possible pathogenetic mechanisms. Acta Haematol 1984;71(2):130-4
    10-28% of the patients with chronic coeliac disease develop a second malignancy, such as non-Hodgkin's lymphomas, particularly of the histiocytic type, and adenocarcinomas. The second tumor may arise in the intestinal tract or in other organs. We describe here a patient who developed a tartrate-resistant, acid phosphatase-positive, hairy-cell leukemia after a history of chronic coeliac disease. In the literature, no similar case has been described as yet.

  5. Gupte SP, et al. Acute myeloid leukemia in a girl with celiac disease. Am J Dig Dis. 1971 Oct;16(10):939-41.

  6. Fundia A, Gomez JC, Maurino E, Boerr L, Bai JC, Larripa I, Slavutsky I Chromosome instability in untreated adult celiac disease patients. Acta Paediatr Suppl 1996 May;412:82-4
    Spontaneous chromosome aberrations (CAs) and induced fragile sites (FSs) were analysed in 12 untreated adult coeliac disease (CD) patients and 8 healthy controls. Blood lymphocytes from each individual were cultured for 72 h at 37 degrees C in F-10 medium with 5% fetal calf serum and 0.1 ml phytohemagglutinine. FSs were induced by FudR (10 micrograms/ml, 24 h before harvesting) and caffeine (2.2 mM. 6 h before harvest). Spontaneous CAs and FSs were analysed on 30-50 Giemsa-stained and G-banded metaphases. The mean frequencies of spontaneous CAs (abnormal cells, gaps/cell and breaks/cell) of CD patients (0.24 +/- 0.02, 0.21 +/- 0.02 and 0.13 +/- 0.02, respectively) were significantly higher than those of controls (0.04 +/- 0.01, 0.02 +/- 0.01 and 0.02 +/- 0.01, respectively) (p < 0.001). Fourteen spontaneous CAs and 5 FSs specific for CD patients presented a strong coincidence (70%) with bands involved in T- and B-cell malignant lymphoma rearrangements. These findings suggest that CD has chromosome instability affecting specific points that could be related to the high prevalence of malignancies in this disorder.

  7. Hsing AW, Hansson LE, McLaughlin JK, Nyren O, Blot WJ, Ekbom A, Fraumeni JF Jr Pernicious anemia and subsequent cancer. A population-based cohort study. Cancer 1993 Feb 1;71(3):745-50