Reversible Hypopituitarism Associated with Intravascular Large B-Cell Lymphoma: Case Report of Successful Immunochemotherapy

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma. There have been only a limited number of reports regarding pituitary dysfunction associated with IVLBCL. We present a 71-year-old woman with hypopituitarism without any hypothalamic/pituitary abnormalities as assessed by magnetic resonance imaging. She presented with edema, abducens palsy, and elevated levels of lactate dehydrogenase and soluble interleukin-2 receptor. Provocative testing showed that the peaks of luteinizing hormone, follicle-stimulating hormone, thyroid-stimulating hormone and adrenocorticotropic hormone were evoked to normal levels by simultaneous administration of luteinizing hormone-releasing hormone, thyrotropin-releasing hormone and corticotropin-releasing hormone, but the responses of these four pituitary hormones showed a delayed pattern. She was diagnosed with IVLBCL with cerebrospinal invasion by pathological findings of the bone marrow, skin, and cerebrospinal fluid. She achieved hematological remission after immunochemotherapy. Pituitary function was also restored without hormonal replacement, and the improvement of the pituitary function was confirmed by dynamic testing. We reviewed the literature with respect to hypopituitarism associated with IVLBCL. There were less than 20 case reports and most of the patients died. Endocrinological course was described in only two cases, and both of them required hormonal supplementation. To our knowledge, this is the first case of hypopituitarism induced by IVLBCL that was successfully managed by immunochemotherapy alone. This case suggests that early diagnosis and treatment of IVLBCL might improve anterior pituitary function and enable patients to avoid hormone replacement therapy.

Intravascular lymphoma with hypopituitarism: A case report

“…Hypopituitarism associated with IVL B-cell lymphoma has been described in fewer than 20 reports[ 3 ]. The pituitary gland is a hypervascular organ, and hypopituitarism may be caused by vascular occlusion by the lymphoid tumor cells in the hypothalamus or pituitary gland[ 3 , 9 ]. It is unclear why selective growth of tumor cells occurs[ 3 , 9 ].…”

“…The pituitary gland is a hypervascular organ, and hypopituitarism may be caused by vascular occlusion by the lymphoid tumor cells in the hypothalamus or pituitary gland[ 3 , 9 ]. It is unclear why selective growth of tumor cells occurs[ 3 , 9 ]. Although we did not perform a pituitary biopsy, we diagnosed lymphoma infiltration of the pituitary gland based on poor evidence of other causes of hypopituitarism and pituitary gland enlargement; both conditions improved after chemotherapy.…”

“…A B-cell immunophenotype is most commonly observed, although cases with T-cell receptor rearrangements have also been reported[ 2 ]. Although various organs can be affected by lymphoma cells, hypopituitarism is an extremely rare complication, and limited cases have been reported[ 3 ]. We herein report a case of hypopituitarism due to IVL, which was successfully treated with chemotherapy.…”

Niedoczynność przysadki u chorego na chłoniaka Hodgkina

“…Wieloosiowa niedoczynność przysadki jest rzadką prezentacją chłoniaka. Opisywano przypadki nacieków chłoniaka rozlanego z dużych komórek B w przysadce [2][3][4]. Znane są także przypadki pierwotnej i jedynej lokalizacji chłoniaków B-komórkowych (zwykle o wysokim stopniu złośliwości) w przysadce [5,6].…”

Intravascular large B‐cell lymphoma presenting as a pituitary mass with bilateral adrenal enlargement and haemophagocytic lymphohistiocytosis

“…The Asian clinical variant of IVLBCL is typified by hepatosplenomegaly, cytopenias and haemophagocytic lymphohistiocytosis (Murase et al , ). Whilst IVLBCL of the endocrine glands has been reported (Matsue et al , ; Sawada et al , ), the presence of simultaneous pituitary and bilateral adrenal gland involvement is exceedingly rare and is associated with a poor prognosis (Matsue et al , ).…”