Endocrine Abstracts

Intravascular Large B-Cell lymphoma (IVLBCL) is an extremely rare type of non-Hodgkin lymphoma involving the growth of lymphoma cells within the vessel lumen without lymphadenopathy. As disease has various modes of presentation and is exceptionally rare, IVLBCL is often diagnosed postmortem. Herein, we report a case of IVLBCL with hypopituitarism, an extremely rare complication, that was successfully treated with chemotherapy. 60 years old woman noticed redness of the abdomen, fatigue, and fever. As she was diagnosed with recurrent erysipelas in the past, it was considered as recurrence of the known infection. Despite antibiotics, disease progressed, redness involved whole abdomen and thighs, fever worsened and swelling of the face and limbs as well as tingling and numbness of the lower extremities, difficulty to walk and acute kidney failure has developed. It was clear that patient did not have an ordinary infection but something else. During next few weeks patient developed severe anasarca (+20 liter), general fatigue, telangiectasis of the trunk, severe polyneuropathy, weight loss and marked panniculitis, she was unable to eat or move independently, mental changes were also notable. As all the possible causes (infections, systemic diseases, etc) were excluded, deep skin biopsy was performed from the abdominal part of the body and IVLBCL was diagnosed. IVLBCL can cause damage of virtually any organ, including endocrine glands, as patient had severe fatigue, anasarca, weight loss and mental changes, pituitary function was carefully evaluated. She was found to have hypopituitarism with central hypothyroidism, central hypogonadism, central adrenal insufficiency, and hyperprolactinemia. IGF-1 was not measured due to technical issues. Brain MRI with contrast showed no tumor or mass in the seller region. It was unclear hypopituitarism was due to intravascular spread of the lymphoma cells in hypophysial portal system and microvascular damage of the pituitary cells, or due to disease severity per se, that is sometimes accompanied with idiopathic hypopituitarism. Patient was started on replacement of hydrocortisone first and only after that levothyroxine was added to treatment regimen. Whole body CT/MRI showed no MTS or lymphadenopathy at all. Chemotherapy with R-CHOP scheme was started with dramatic improvement of patient’s clinical and laboratory parameters. Hypopituitarism was resolved after 3 months. After 8 course of chemotherapy patient progressed with new skin nodules all over the body, however before starting the second line Chemotherapy, patient started to go into spontaneous remission, nodules disappeared without any treatment at all and she is in remission until now.