First Reported Case of Lymphomatoid Granulomatosis Associated with Azathioprine Therapy in Crohnʼs Disease
Bibliographic record
Abstract
Background: Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV) induced lymphoproliferative disorder associated with immunodeficiency. Case: A 26-year-old woman presented with syncope after 3 days of fever, RUQ pain and malaise. She had a 6-year history of Crohn colitis initially managed with an oral 5-ASA product but started on azathioprine (2.5 mg/kg) 15 months ago due to loss of response. Examination revealed tender hepatomegaly. Investigations revealed pancytopenia (Hb=119 g/L, WBC=1.4 × 109/L, plts=80 × 109/L), elevated unconjugated bilirubin (29 umol/L) and INR (1.8). All cultures were negative. Ultrasound and MRI only showed hepatomegaly. Six weeks later, repeat MR abdomen showed extensive T1-hyperintense foci ranging from 1-8 cm in diameter distributed throughout the liver, lung bases and kidneys bilaterally. CT of the chest further delineated peripheral parenchymal lung lesions. Ongoing presyncopal symptoms prompted brain imaging, also revealing lesions in the cortex and cerebellum consistent with small vessel infarcts of varying ages. An autoimmune and vasculitis work-up was negative. EBV serology and PCR were negative. Core liver biopsy and bronchoalveolar lavage revealed atypical B-lymphocytes. The liver biopsy showed angiocentric lymphoid infiltration but negative studies for EBV-encoded small RNA (EBER). The patient then developed worsening dyspnea and an empyema requiring drainage. A tharocoscopic lung biopsy was performed and histopathology demonstrated an angiocentric lymphoid infiltrate characterized by atypical large CD20+ B-cells that stained EBER-positive clinching the diagnosis of LYG. Treatment was initiated with recombinant interferon-alpha titrated up to 10 million units per m2. Four doses of rituxumab 375 mg/m2 were also administered. On therapy, her symptoms have resolved and the brain, lung, kidney and liver lesions are all steadily regressing. Conclusions: LYG is one of several EBV driven lymphoproliferative disorders that requires a high index of suspicion in immunocompromised patients. This is the first report to our knowledge of LYG developing in a patient with Crohn disease on azathioprine therapy. LYG is graded I-III depending on the degree of EBER-positivity and organ involvement. Grades I-II usually follow an indolent course but may require immunotherapy in addition to withdrawal of immunosuppression. Grade III LYG follows an aggressive course and requires multi-agent chemotherapy; it acts like a acts like a large B-cell lymphoma and carries a poor prognosis.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".