THINK PORPHYRIA: CASE REPORT AND REVIEW OF LITERATURE.

Porphyrias are a group of rare disorders caused by enzyme defects in haem biosynthesis pathway. Acute intermittent porphyria is the most common hepatic porphyria. The disorder presents with severe neuropathic abdominal pain that can be accompanied by a wide range of gastrointestinal, psychiatric and neurological symptoms, making the diagnosis clarification very challenging. We report a case of a 27-year-old female patient who presented with acute abdominal pain, vomiting and marked hyponatremia, developed seizures and disorientation, and eventually required intensive care unit treatment to maintain breathing. Her symptoms were initially misinterpreted as a functional gastrointestinal disorder, thus delaying the needed specific treatment. She was diagnosed a week after the initial hospital admission, and her condition improved after receiving treatment with intravenous glucose and haemin. For patients with acute neurovisceral attacks, early clinical recognition is essential. Severe hyponatremia, urine that develops orange colour on exposure to light and gastrointestinal symptom combination with neurologic symptoms are three valuable clues that may lead to the right diagnosis faster. Pathophysiology of hyponatremia in case of acute intermittent porphyria in only partly understood and can be associated with syndrome of inappropriate antidiuretic hormone secretion, gastrointestinal or renal sodium loss.

Rare Case of Hodgkin Lymphoma Transformation into Diffuse Large B-Cell Lymphoma with Atypical Spread Epidurally, Intradurally and Intramedullary: A Case Report.

BACKGROUND Hodgkin lymphoma (HL) transformation into diffuse large B-cell lymphoma (DLBCL) is uncommon, and scant information has been published on transformed high-grade lymphomas. Therefore, it is important to present and discuss cases of lymphoma transformation to make new information on disease progression, diagnosis, and treatment more readily available. In this paper, we present a case of HL transformation into DLBCL with atypical dissemination. CASE REPORT A 39-year-old woman presented with severe hip pain. A computed tomography (CT) scan was performed, which showed massive pathological retroperitoneal and pelvic lymphadenopathy. The lymph nodes were biopsied and revealed HL. The patient then underwent 7 cycles of ABVD therapy; however, clinical concern was raised for persistent disease due to the poor response to therapy. A vertebral body biopsy was performed to clarify the diagnosis, and histological analysis revealed DLBCL. Therefore, specific chemotherapy with the R-CHOP scheme was begun; the patient received 8 cycles of rituximab and residual lymphoma tissue irradiation. Two months later, magnetic resonance imaging later demonstrated radiological disease progression with multiple widespread metastases in the spinal vertebrae as well as prevertebral, epidural, intradural, and intramedullary metastatic spread. The patient underwent intrathecal chemotherapy and radiation therapy, after which, full metabolic remission was observed on PET/CT. CONCLUSIONS Vigilance should be maintained for patients with poor response to HL treatment owing to the possible transformation into DLBCL. However, even in such cases, full metabolic remission can be achieved with appropriate treatment.