Panhypopituitarism As The Initial Manifestation Of Diffuse Large B-cell Lymphoma: A Case Report And Literature Review

Objective: To investigate the clinical characteristics,diagnosis and therapy of the Diffuse Large B-cell Lymphoma with dyspituitarism as the initial manifestation,so as to improve the understanding of the disease,and promote the early diagnosis and treatment efficiency.Methods: We retrospectively reviewed a case of Diffuse Large B-cell Lymphoma with panhypopituitarism as the initial manifestation,and diagnosed in the First Affiliated Hospital of Guangxi Medical University in 2019.Relevant literatures with dyspituitarism as the initial manifestation of Diffuse Large B-cell Lymphoma were retrieved,and we summarized and analyzed the clinical characteristics and diagnostic methods of the disease.Results: 1.Data in this case: Female patient,56 years old.Starts with symptoms of fatigue,anorexia,headache,followed by chills,fever,and weight loss.Laboratory examination showed hypopituitarism,anemia,leukopenia,and blood lactate dehydrogenase increased significantly.Afterwards,pancytopenia,hypofibrinogenemia,and liver function impairment gradually appeared.Imaging examination showed adenohypophysis occupation,exudative lesions of both lungs,multiple enlarged mediastinal lymph nodes,and hepatosplenomegaly.PETCT showed increased glucose metabolism in left supraclavicular lymph nodes,multiple mediastinal lymph nodes,liver,spleen,and thoracic vertebral bone marrow.Mediastinal lymph nodes and liver biopsy: under the microscope,a large number of medium to large heterotypic cells were seen in diffuse infiltration,and the mitosis was easy to see.Immunohistochemistry: CD20(+),CD79a(+),CD5(+),CD10(-).FISH: all were negative for bcl-2,bcl-6,and MYC.Diagnosis: CD5-positive Diffuse Large B-cell Lymphoma(non-germinal center source).R-CHOP chemotherapy is effective.2.Systematic literature review results: 13 cases of Diffuse Large B-cell Lymphoma presented with initial symptoms of pituitary dysfunction were reported from 2000 to 2019.A total of 14 cases including the present case.1.Clinical manifestation: anorexia(43%),fatigue(43%),more Polyuria and polydipsia(36%),digestive(29%),extraocular myoparalysis(36%)caused by pituitary function impairment and pituitary placeholder,fever(36%),weight loss(36%)and superficial lymph node enlargement(14%)gradually or at the same time.2.Endocrine function examination showed adenohypopituitarism(100%)and central diabetes insipidus(50%).Other laboratory abnormalities included increased lactate dehydrogenase(43%),hematocytopenia(50%),hyponatremia(29%)and increased blood sedimentation(29%).3.Pituitary MRI mainly for saddle area tumors(50%),pituitary stalk thickening(42%),the pituitary gland density change(25%),CT showed deep lymph node enlargement(67%),adrenal tumors(67%),liver nodules(33%)and splenomegaly(17%),PET-CT affected areas are mainly the pituitary gland,adrenal gland,lymph nodes,spleen,liver,bone,bone marrow.4.Diffuse Large B-cell Lymphoma was confirmed by pathological biopsy in all cases,and pituitary infiltration was confirmed by saddle region biopsy in 3 cases.The main biopsy methods were percutaneous biopsy(44%),surgical resection(33%),and endoscopic transnasal saddle biopsy(22%).5.Treatment and prognosis: After chemotherapy based on CHOP(cyclophosphamide,adriamycin,vincristine,prednisone)and hormone replacement therapy,6 patients died,and the Overall survival time was 1.4-12.7months.8 patients were still alive at the time of case report,and the follow-up time was 4.2-48 months.Among them,pituitary lesions disappeared or responded in 6 cases,and pituitary endocrine function improved in 2 cases.Conclusion: 1.DLBCL with pituitary dysfunction as the first manifestation is rarely observed,mostly in the middle-aged and elderly male patients.2.The onset of the disease is insidious.and the most common early symptoms are anorexia and fatigue caused by hypopituitarism,laboratory tests show hemocytopenia,elevated lactate dehydrogenase.The disease is widely affected,except for the pituitary,the most frequently affected sites are deep lymph nodes and adrenal glands.3.DLBCL pituitary infiltration is mainly manifested as saddle area mass,thickening of pituitary stalk and change of pituitary density.When the anterior and posterior lobes of the pituitary are involved,they often invade the optic chiasm and hypothalamus.4.CD20 positive is a typical immunophenotype of DLBCL,which has diagnostic value,pituitary infiltration of DLBCL mainly relies on clinical characteristics and the effect of chemotherapy for exclusion diagnosis.5.The treatment regimen was mainly R-CHOP chemotherapy combined with hormone replacement therapy.The structural and function of the pituitary gland in most patients can be recovered after treatment.