Contributions
Abstract: PB1803
Type: Publication Only
Background
RCD is defined by no response to a year of gluten free diet (GFD) with persistence of malabsorption and intestinal villous atrophy. A biopsy of upper gastrointestinal tract is necessary for diagnosis of the two RCD subtypes: type I characterized by a normal phenotype of intraepithelial lymphocytes (IEL) with surface CD3 and CD8 expression; type II characterized by clonal expansion of abnormal IEL lacking surface markers CD3, CD8 and T-cell receptor and preserved intracellular CD3 expression. RCD II has a severe clinical presentation due to ulcerative jejunitis responsible for severe protein loss enteropathy and malnutrition. The risk of transformation in enteropathy associated T cell lymphoma (EATL) is about 33-52% within 5 years after diagnosis. Only 30-40% of patients (pts) achieve histological response and in most cases the clinical improvement is transient with steroids. Immunosuppressive therapy has failed to show a histological response, even increasing the risk of evolution in EATL. Cladribine, a purine analogue, at the dosage of 0.14 mg / kg per day for 5 days has induced clinical and histological response.
Aims
To achieve an early and accurate diagnosis of RCD type II pts and to evaluate clinical and histological response to purine analogues treatment.
Methods
We retrospectively collected clinical, laboratory, endoscopic end histological data of two RCD type II pts treated with subcutaneous (SC) cladribine from 2015 in our haematology unit.
Results
The first pt was a 63 years old man diagnosed with CD since 2007. In 2012 he underwent esophagogastroduodenoscopy (EGD) for diarrhea and weight loss with diagnosis of RCD I. A steroid therapy was started with transient clinical benefit. In July 2015, a new EGD was performed for recurrence of symptoms and showed a 60% T-IEL infiltrate with aberrant phenotype and a monoclonal TCR on a polyclonal basis. Video capsule endoscopy (VCE) demonstrated widespread atrophy in more than half of the small intestine, while CT scan and PET resulted negative. Because of steroid refractoriness, a treatment with SC cladribine was started. He underwent three cycles every six months, with a 30% dose reduction for the first two cycles. Treatment was well tolerated, without hematological toxicity. After III cycles he had weight gain and resolution of diarrhea and a macroscopic improvement of ileal athrophy and ulcers, without significant reduction of T cell infiltration.
The second pt was a 62 years old woman diagnosed with CD in January 2016. A EGD revealed a 60% T-IEL infiltrate with aberrant phenotype and monoclonal TCR on a polyclonal basis. VCE demonstrated widespread signs of jejunal atrophy and erosions, while CT scan and PET were negative. Since steroids were ineffective, she underwent two cycles of SC cladribine. Treatment was well tolerated. There was an improvement of clinical symptoms (weight gain and resolution of diarrhea) and of ileal atrophy and ulcers, with a 50% reduction T IEL infiltrate.
Conclusion
In conclusion both pts showed a clinical and histological response to SC cladribine without hematologic toxicity or infectious complications: this indicate that this treatment could be a safe and effective option in elderly pts affected by type II RCD. Furthermore the outpatient administration of the treatment also lead to a reduction of pt discomfort and costs related to hospitalization.
Session topic: 21. Aggressive Non-Hodgkin lymphoma - Clinical
Keyword(s): Cladribine, Elderly, Lymphoid malignancy, subcutaneous
Abstract: PB1803
Type: Publication Only
Background
RCD is defined by no response to a year of gluten free diet (GFD) with persistence of malabsorption and intestinal villous atrophy. A biopsy of upper gastrointestinal tract is necessary for diagnosis of the two RCD subtypes: type I characterized by a normal phenotype of intraepithelial lymphocytes (IEL) with surface CD3 and CD8 expression; type II characterized by clonal expansion of abnormal IEL lacking surface markers CD3, CD8 and T-cell receptor and preserved intracellular CD3 expression. RCD II has a severe clinical presentation due to ulcerative jejunitis responsible for severe protein loss enteropathy and malnutrition. The risk of transformation in enteropathy associated T cell lymphoma (EATL) is about 33-52% within 5 years after diagnosis. Only 30-40% of patients (pts) achieve histological response and in most cases the clinical improvement is transient with steroids. Immunosuppressive therapy has failed to show a histological response, even increasing the risk of evolution in EATL. Cladribine, a purine analogue, at the dosage of 0.14 mg / kg per day for 5 days has induced clinical and histological response.
Aims
To achieve an early and accurate diagnosis of RCD type II pts and to evaluate clinical and histological response to purine analogues treatment.
Methods
We retrospectively collected clinical, laboratory, endoscopic end histological data of two RCD type II pts treated with subcutaneous (SC) cladribine from 2015 in our haematology unit.
Results
The first pt was a 63 years old man diagnosed with CD since 2007. In 2012 he underwent esophagogastroduodenoscopy (EGD) for diarrhea and weight loss with diagnosis of RCD I. A steroid therapy was started with transient clinical benefit. In July 2015, a new EGD was performed for recurrence of symptoms and showed a 60% T-IEL infiltrate with aberrant phenotype and a monoclonal TCR on a polyclonal basis. Video capsule endoscopy (VCE) demonstrated widespread atrophy in more than half of the small intestine, while CT scan and PET resulted negative. Because of steroid refractoriness, a treatment with SC cladribine was started. He underwent three cycles every six months, with a 30% dose reduction for the first two cycles. Treatment was well tolerated, without hematological toxicity. After III cycles he had weight gain and resolution of diarrhea and a macroscopic improvement of ileal athrophy and ulcers, without significant reduction of T cell infiltration.
The second pt was a 62 years old woman diagnosed with CD in January 2016. A EGD revealed a 60% T-IEL infiltrate with aberrant phenotype and monoclonal TCR on a polyclonal basis. VCE demonstrated widespread signs of jejunal atrophy and erosions, while CT scan and PET were negative. Since steroids were ineffective, she underwent two cycles of SC cladribine. Treatment was well tolerated. There was an improvement of clinical symptoms (weight gain and resolution of diarrhea) and of ileal atrophy and ulcers, with a 50% reduction T IEL infiltrate.
Conclusion
In conclusion both pts showed a clinical and histological response to SC cladribine without hematologic toxicity or infectious complications: this indicate that this treatment could be a safe and effective option in elderly pts affected by type II RCD. Furthermore the outpatient administration of the treatment also lead to a reduction of pt discomfort and costs related to hospitalization.
Session topic: 21. Aggressive Non-Hodgkin lymphoma - Clinical
Keyword(s): Cladribine, Elderly, Lymphoid malignancy, subcutaneous