Passenger lymphocyte syndrome (PLS) is a well-described phenomenon causing immune hemolytic anemia, mostly in non-ABO identical transplantations. The syndrome occurs when donor lymphocytes produce antibodies against the recipient's red blood cells. Although the syndrome is usually self-limited, further management with blood transfusions, immunosuppression, or plasmapheresis might be needed. A 23-year-old female with AB
+
blood group underwent small intestine transplantation from a deceased donor with O
+
blood group. She received rituximab, thymoglobin, and methylprednisolone as immunosuppressive induction. In the 9
th
postoperation day, she developed hemolysis which was primarily managed with blood transfusions and finally ceased by plasmapheresis and intravenous immunoglobulin. Few cases of PLS have been previously described in intestinal transplantation recipients. Correct diagnosis and management prevents severe hemolysis outcomes. Previous cases have been successfully treated with a combination of immune suppression, plasma exchange, and transfusions.
Severe acute respiratory syndrome coronavirus-2 (SARS-COV-2) that is known as COVID-19 is a new emerging respiratory infection attributed to novel coronavirus, firstly introduced in Wuhan, China, at the end of 2019. This infection is still of great concern because of various presentations of the disease, which are not fully understood. The manifestations of this virus among liver transplanted patients would be more challenging in the setting of immunosuppression. The focus of this study is to introduce different presentations of this virus in five liver transplant recipients referred to the gastroenterology ward of Taleghani Hospital, a teaching referral hospital in Tehran, Iran. These patients were started on different types of therapies for coronavirus infection, from only supportive care up to remdisivir infusion and hemoperfusion based on the severity of the disease. Additionally, they were advised to continue all their immunosuppressant agents with adjustment except for CellCept that was withheld.
Background
ACTH-independent macronodular hyperplasia (AIMAH) is an uncommon disorder characterized by massive enlargement of both adrenal glands and hypersecretion of cortisol. Concomitant AIMAH and multiple endocrine neoplasia type1 (MEN1) is rare to our knowledge.
Case presentation
Herein, we describe a 32 year old woman with long history of prolactinoma and secondary ammonhrea presented with not-severe manifestation of hypoglycemia due to concomitant presence of insulinoma with AIMAH leading to 12 years delay of MEN1 diagnosis. Laboratory tests showed severe hypoglycemia associated with hyper insulinemia (non-fasting blood sugar = 43 mg/dl, insulin = 80.6 μIU /ml, C-peptide = 9.3 ng/ml) hyperparathyroidism (calcium = 10.3 mg/dl, phosphor = 3.1 mg/dl, PTH = 280 pg/ml) and chemical evidence of an ACTH-independent hypercortisolism (serum cortisol value of 3.5, after 1 mg dexamethasone suppression test serum ACTH value of 17 pg/ml, and high urinary cortisol level). Abdominal CT scan demonstrated two enhancing well-defined masses 27*20 mm and 37*30 mm in the tail and body of the pancreas, respectively, and a 36*15 mm mass in left adrenal gland (seven Hounsfield units). Dynamic pituitary MRI revealed a partial empty sella. The physical examination of the patient was unremarkable. Distal pancreatectomy and a left adrenalectomy were performed. After the surgery, we observed clinical and biochemical remission of hyper insulinemia and gradual decrease in urinary cortisol. The histological features of the removed left adrenal gland were consistent with AIMAH. Histological examination of the pancreatic lesions revealed well differentiated neuroendocrine tumors. Genetic abnormalities in the MEN1, heterozygote for pathogenic variant chr11; 645,773,330-64577333AGAC, c.249-252delGTCT, p. (11e85Serfs Ter33) in exon 2 were found. It was recommended the patient undergoes parathyroidectomy as soon as possible.
Conclusion
Given the history and presentation of our case, we recommend that the clinicians consider the possibility of autonomous cortisol production in MEN1 patients who do not show severe symptoms of hypoglycemia in the presence of insulinoma.
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