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Guarina A. et al: Association of Immune Thrombocytopenia and Celiac DiseaseTurk J Hematol 2021;38:175-180AbstractResults: The first diagnosis was ITP in 57.1% and CD in 32.1% ofpatients. In 3 patients (10.7%), the two diagnoses were simultaneous.All the potential and silent cases of CD in our cohort were diagnosedin the groups of “ITP first” and “simultaneous diagnosis”. In all childrenITP was mild, and in 2 out of 8 not recovered from ITP at the timeof CD diagnosis a normalization of platelet counts (>100,000/µL)occurred 3 and 5 months after starting a gluten-free diet, respectively.Conclusion: We think that screening for CD should be consideredin children with ITP regardless of the presence of gastrointestinalsymptoms. Furthermore, some patients may recover from ITP afterstarting a gluten-free diet.Keywords: Celiac, Children, Immune, Thrombocytopenia, PediatricÖzBulgular: Hastaların %57,1’inde ilk tanı İTP ve %32,1’inde ÇH idi.Üç hastada (%10,7) iki tanı aynı anda konulmuştu. Kohortumuzdakitüm potansiyel ve sessiz ÇH olguları “önce İTP” ve “eşzamanlı tanı”gruplarında teşhis edildi. Tüm çocuklarda İTP hafifti ve ÇH tanısısırasında trombositopenisi devam eden 8 çocuktan 2’sinde, glutensizdiyete başladıktan sonra sırasıyla 3 ve 5 ayda trombosit sayılarındanormalleşme (>100.000/µL) görüldü.Sonuç: Bu çalışma İTP’li çocuklarda gastrointestinal semptomlarınvarlığına bakılmaksızın ÇH taramasının yapılması gerektiğinidüşündürmektedir. Bazı hastalarda trombosit sayılarının glutensiz birdiyete başladıktan sonra düzelebileceği akılda bulundurulmalıdır.Anahtar Sözcükler: Çölyak, Çocuklar, İmmün, Trombositopeni,PediatrikIntroductionCeliac disease (CD) is an immune disorder of the small intestine,often associated with other autoimmune disorders [1], whoseprevalence has been estimated in Europe as 0.7% [2]. Immunethrombocytopenia (ITP) is an acquired disease caused byautoantibodies against platelet antigens whose estimated yearlyincidence in the pediatric population is 0.0019%-0.0064% [3].The features of ITP and CD coexistence have been investigatedin some cohorts and case reports with contradictory results,especially regarding the efficacy of a gluten-free diet on ITP [4,5,6,7,8,9,10,11,12,13,14,15,16,17,18]. Furthermore, no study hasaimed to describe the specific features of this association in thepediatric population. The goal of this study was to characterizethe association of these two diseases in Italian children.Materials and MethodsA case report form (CRF) was sent to all 55 AIEOP (AssociazioneItaliana Emato-Oncologia Pediatrica) Centers collectinginformation about patients up to 18 years of age with both CDand ITP diagnosed from January 1, 2000, to December 31, 2019.Children affected by a known immunodeficiency were excluded.The clinical phenotypes of ITP and CD autoantibody testing (antitransglutaminaseantibodies ± anti-endomysial antibodies) ±biopsy results were recorded. CD was considered and classifiedaccording to Tonutti and Bizzaro [19] as classic (presence ofsymptoms of malabsorption, positive CD-specific antibodies andbiopsy, and symptom resolution with a gluten-free diet), silent(positive CD-specific antibodies, HLA DQ2 or DQ8, and biopsywithout symptoms), latent (presence of HLA DQ2 or DQ8 andhistological alterations typical of CD in duodenal biopsy at somepoint in life, but neither symptoms nor positive antibodies), orpotential (positive CD-specific antibodies and HLA withouthistological abnormalities in duodenal biopsies). ITP was definedas newly diagnosed (within 3 months from diagnosis), persistent(between 3 and 12 months from diagnosis), or chronic (cITP,lasting for more than 12 months) [20]. The ITP bleedingscore was defined as follows [21]: Type A: asymptomaticpaucisymptomaticITP, with clinical symptoms ranging from nobleeding to a few petechiae and some bruises without mucosalhemorrhages; Type B: intermediate ITP, a clinical picture withmore petechiae, bruising, and mucosal hemorrhages; Type C:severe ITP, a clinical picture with severe cutaneous and mucosalbleeding symptoms with at least one of the following: retinalhemorrhages, intracranial hemorrhage, other severe internalhemorrhages, hemorrhagic shock, or life-threatening bleeding.The timing of the diagnosis of the patients was classified asfollows: 1) CD first: CD was diagnosed before ITP; 2) ITP first: ITPwas diagnosed before CD; 3) Simultaneous diagnosis: the seconddisease (CD or ITP) was diagnosed during hospitalization orinitial ascertainment for the other disorder. Considering therapyor efficacy of a gluten-free diet on ITP, complete response wasdefined as any platelet count of ≥100,000/µL with the absenceof bleeding, partial response as any platelet count between30,000 and 100,000/µL with at least doubling of the baselinecount and absence of bleeding, and no response as any plateletcount of <30,000/µL or less than doubling of the baseline countand/or occurrence of bleeding.Statistical AnalysisStatistical analysis was performed using open source R statisticalsoftware [22].ResultsWe collected CRFs for 28 ITP/CD patients diagnosed in Italy from2000 to 2019; they were all Caucasian (male/female=1/2.1).Among CD patients, for 11 the diagnosis was performedthrough intestinal biopsy + anti-transglutaminase ± endomysialantibodies detection, and for the remaining 17 throughantibody dosages only according to ESPGHAN guidelines. Themost relevant clinical features of the cohort are shown in176
Turk J Hematol 2021;38:175-180Guarina A. et al: Association of Immune Thrombocytopenia and Celiac DiseaseTable 1. The median follow-up (FUP) time from ITP and CDdiagnosis was 4.4 years (range: 0.2-16.5) and 2.3 years (range:0.02-15.2), respectively. Median age at CD and ITP diagnosis was6.2 and 5.8 years, respectively. Among 27 evaluable patients,a positive family history for other autoimmune diseases waspresent in 15 (55.6%): thyroid disorders were reported in 66.6%of these cases and CD in 40%. ITP was present in the familyhistory of 1 patient only (grandfather). Extraintestinal disordersaffected 4 patients (14.8%); interestingly, 3 out of 4 wereautoimmune hemolytic anemia. The median number of plateletsat ITP diagnosis was 19,500/µL (range: 1,000-96,000) and thebleeding score was A in 67.9% and B in 32.1% of patients,respectively. First-line therapy for ITP was IVIG in 18 patients(64.3%) and oral prednisone in 4 patients (14.3%), while 6patients (21.4%) were not treated (“wait and see”). ITP turnedout to be persistent in 12 out of 27 evaluable cases (44.4%) andchronic in 10 out of 25 evaluable cases (40%); the median numberof platelets at 3 and 12 months from ITP onset was 100,000/µL(range: 1,000-446,000) and 135,000/µL (range: 15,000-343,000),respectively; only 1 patient at 12 months from ITP diagnosishad platelets of >20,000/µL. At the last FUP, 25.0% of patientswere still presenting with cITP; none of them had platelets of>50,000/µL and only 1 patient was still on treatment (withmycophenolate mofetil). Helicobacter pylori was tested in 57.1%of patients using a stool antigen test and was found negative inall cases. Bone marrow aspiration was performed for 57.1% ofpatients and was always consistent with ITP. CD was classifiedas classical, silent, latent, and potential in 17 (60.7%), 7 (25%),2 (7.1%), and 2 (7.1%) cases, respectively. The first diagnosiswas ITP for 16 patients (57.1%) and CD for 9 patients (32.1%).For 3 patients (10.7%), the two diagnoses were simultaneous; 1potential and 2 classical cases of CD were discovered just afterITP onset, in 1 patient after having performed an autoimmunepanel for screening and in the other 2 after having evaluatedpre-existing gastrointestinal symptoms. The most relevantfeatures of the “CD first” and “ITP first” patients are shownin Table 2. In the “CD first” subgroup (9 patients), the mediantime from diagnosis of CD to diagnosis of ITP was 1.6 years(range: 0.2-10.1). These 9 cases of CD were all of the classic type,but 2 (latent) patients were on a gluten-free diet at the momentof ITP diagnosis. None of them suffered from other autoimmunediseases and 3 out 8 evaluable patients (37.5%) developedcIPT. In the “ITP first” subgroup (16 patients), the mediantime from diagnosis of ITP to diagnosis of CD was 2.8 years(range: 0.1-12.9) and all cases of silent CD (n=7), diagnosedthanks to the autoimmune screening performed in the centers,Table 1. Characteristics of the patients.Median age at CD diagnosis (years) 6.2 (range: 0.6-27.6)Median age at ITP diagnosis (years) 5.8 (range: 0.5-15.1)Median PLTs at ITP diagnosisFamily history of autoimmune diseases 55.6%Extraintestinal disorders 14.8%CD typeITP bleeding scoreTiming of diagnosisEfficacy of gluten-free diet on ITPcITP at 12 months 40%First-line therapy for ITP19,500/µL (range: 1,000-96,000/µL)Latent: 7.1%Potential: 7.1%Silent: 25%Classic: 60.7%Score A: 67.9%Score B: 32.1%ITP first: 57.1%CD first: 32.1%Simultaneous: 10.7%NR: 77.8%CR: 22.2%First rescue therapy for ITP 42.9%Second rescue therapy for ITP 21.4%IVIG: 64.3%Wait and see: 21.4%Oral prednisone: 14.3%Median FUP from ITP diagnosis (years) 4.4 (range: 0.2-16.5)Median FUP from CD diagnosis (years) 2.3 (range: 0.02-15.2)CD: Celiac disease; ITP: immune thrombocytopenia; FUP: follow-up; PLTs: platelets; NR: no response; CR: complete response; cITP: chronic immune thrombocytopenia; IVIG: intravenousimmunoglobulin.177
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