33-year-old woman referred for abdominal pain. Allergic to penicillin, tonsillectomy and adenoidectomy, she had a normal pregnancy with normal delivery 4 years ago, and reported no toxic habits or regular medication. She came to the emergency department with pain in the left hypochondrium for 3 months, intensified in the last 24 hours. She also presented with increased abdominal perimeter, nausea and fever of 38.5 ºC, with no other infectious focality. No toxic syndrome or altered bowel habits were reported.
On examination, she was conscious, oriented, normohydrated, with some pallor of the skin and mucous membranes. BP 140/80, HR 100 bpm, RR 20 rpm, axillary temperature 37.3 ºC. Cardiac and pulmonary auscultation normal. The abdomen is soft, depressible, with the only finding being a hard, painful splenomegaly of about 17 cm. No peripheral lymph nodes were palpable and the extremities were normal.
Laboratory tests on admission showed 2,900 leukocytes/mm3 (82% N, 14% L), HB 8.2 g%, HtH 26%, MCV 84.8, RDW 17.1, 79,000 plaq/mm3, quick 66%, cephalin 29; other biochemistry parameters were strictly normal (glycaemia, renal function and ionogram). Radiologically, there was elevation of the two haemidiaphragms without alteration of the pulmonary hilarity, and displacement of the intestinal loops due to a mass effect in the left hypochondrium.
With a diagnosis of splenomegaly and pancytopenia, she was admitted to haematology. Abdominal ultrasound showed images compatible with multiple intraparenchymal splenic haematomas. The thoracoabdominal CT scan showed no other findings. Cytogenetic studies were negative, viral hepatic serology negative, mantoux negative, normal hormonal study, normal folic acid metabolites, complete coagulation study with no other findings than thrombopenia, normal haptoglobin, negative erythrocyte polyspecific direct coombs, proteinogram with slightly increased gamma globulin (22.4%; normal < 18.6%) and slightly decreased iron, 25 microg% (normal < 37). Tumour markers were negative, as well as screening for occult tumours (mammography, colonoscopy, genital cytology, bone marrow biopsy). During this study, she required the transfusion of 450 cc of de-leukocyte red blood cell concentrate, and remained haemodynamically stable, with abdominal pain controlled with first-stage analgesics.
With the diagnosis of hypersplenism syndrome, a splenectomy was requested, which was performed laparoscopically without incident. The postoperative period was afebrile, without local or general complications, afebrile, pain remission, recovery of oral intake and removal of the drain.
The anatomopathological report of the specimen showed: splenic peliosis without endothelial hyperplasia, with sinus histiocytosis and macrophagic cells and haemosiderin crystals.
After one year of follow-up, the woman has recovered the normal parameters of the three haematological series without any treatment, with no evidence of complications or hepatic recurrence.