H AIRY CELL leukemia, a disease characterized by splenomegaly without adenopathy, by pancytopenia, and by circulating malignant cells with prominent cytoplasmic projections requires therapy in most cases.”2 The manifestations of hairy cell leukemia result from a balance between (I) leukemic infiltration of the red pulp of the spleen by hairy cells, which leads to hypersplenism,3 and (2) leukemi...

Splenomegaly, sometimes of massive extent, occurs in a large number of hereditary diseases, some relatively prevalent and others, rare to ultra-rare. Because physicians are often unfamiliar with the less common disorders, patients may suffer because of diagnostic delay or diagnostic error and may undergo invasive, non-innocuous procedures such as splenectomy that are potentially avoidable were ...

Wilson disease (WD), referred to as hepatolenticular degeneration and occurring primarily as neurological and liver disease, is an inherited disorder that has various clinical presentations [1–3]. The altered gene is localized on the long arm of chromosome 13. Mutations in the Wilson gene are common and include small insertions or deletions. In the cases of unidentified tubular dysfunctions, he...

BACKGROUND Idiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia. This study evaluates the pathogenic concept of the disease by a systematic review of the literature and illustrates novel pathologic and laboratory findings. CASE PRESENTATION We report the first ...

Management of a patient with portal hypertension and hypersplenism who is scheduled for elective surgery is a challenge for anesthesia providers. We report a case that was scheduled for elective surgery in which preoperative splenic artery embolization improved the hematologic parameters. A 32-year-old woman was scheduled for total abdominal hysterectomy for multiple fibroids and menorrhagia wi...

In 1898 Banti described a disorder characterized by splenomegaly and hypersplenism, resulting in portal hypertension and anemia in the absence of hematological disease. 1 Banti's syndrome is also known as non-cirrhotic portal hypertension (NCPH) in India and Idiopathic Portal Hypertension (IPH) in Japan. Hepatoportal sclerosis seems to be its counterpart in the United States. 2,3 Banti's syndro...

BACKGROUND The clinico-surgical implication and successful management of a rare case of factor five (V) deficiency with portal hypertension and hypersplenism due to idiopathic extra-hepatic portal venous obstruction is presented. CASE PRESENTATION A 16-year old boy had gastro-esophageal variceal bleeding, splenomegaly and hypersplenism. During preoperative workup prolonged prothrombin time an...

BACKGROUND Single incision laparoscopic surgery (SILS) is an emerging technique that has been used as an approach for appendectomy, cholecystectomy, and splenectomy. We describe the technique of single incision laparoscopic splenectomy for hypersplenism in a 5-year-old boy with spherocytosis. CASE REPORT The patient required blood transfusions for anemia secondary to hypersplenism. His spleen...