Caroli disease is a rare congenital malformation that predisposes to segmental cystic dilatation of the intrahepatic bile ducts. Banti syndrome is characterized by persistent splenomegaly due to chronic congestion, resulting in a low hematocrit and ultimately leading to pancytopenia. In this report, we describe a 29-year-old woman who presented with a >20-year history of hepatitis B surface antigen positivity and a >1-year history of recurrent fatigue and malaise. On examination, the patient had abdominal distension with marked splenomegaly (7 cm below the ribs) and ascites with tenderness of the abdominal muscles to palpation. A complete blood count showed a low white blood cell count, red blood cell count, and hemoglobin concentration. During the course of treatment, the patient developed multiple symptoms of pancytopenia and concomitant splenomegaly, and she was discharged after total splenectomy with good recovery. The combination of Banti syndrome and Caroli disease results in severe symptoms of portal hypertension.

BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma. Patients usually present with splenomegaly and pancytopenia but without lymphadenopathy. Immunohistochemistry (IHC) staining of bone marrow biopsy shows intra-sinusoidal infiltration of CD3 and CD56 T-lymphocytes. Current treatment strategy of HSTCL includes a CHOP regimen (cyclophosphamide, adriamycine, vincristine, prednisone) followed by autologous transplantation.
METHODS: A 28-year-old male presented with abdominal fullness, weight loss, and massive splenomegaly. Laboratory findings revealed pancytopenia. A CT scan of the abdomen displayed hepatomegaly and massive splenomegaly. The bone marrow pathology examination showed monotonous medium-sized lymphocytes with some cluster of atypical lymphocytes with loosely condensed chromatin and pale cytoplasm. The intra-sinusoidal location was more prominent after using IHC staining of CD3 and CD56, which are characteristics of HSTCL. We administered CHOP-based regiment every 3 weeks for 3 cycles; however, the response was a stable disease. Since the splenomegaly was still massive and compromised the patient, the multidisciplinary team decided to perform splenectomy. Unfortunately, the patient did not survive the surgery.
CONCLUSIONS: Hepatosplenic T-cell lymphoma is a rare aggressive disease, which is part of peripheral T-cell lymphoma. CHOP-based chemotherapy appeared to be ineffective, and we need further studies to find the optimal treatment of HSTCL.

Scrub typhus is a serious public health problem in the Asia Pacific region. Early diagnosis and treatment can prevent complications and mortality. Splenomegaly in isolated scrub typhus is mostly mild or subclinical and rarely massive. We report an adolescent boy with scrub typhus presenting with fever, massive splenomegaly and severe anemia. Clinicians should be aware of atypical presentations of scrub typhus, which is easily treatable with minimal investigations.

Gaucher disease (GD) is a rare, autosomal recessive genetic disease caused by deficiency of a lysosomal enzyme (glucocerebrosidase/β-glucosidase) that leads to the accumulation of its substrate in lysosomal macrophages. GD remains rare and delayed diagnosis is common due its gradual onset. It is important to include this differential diagnosis in cases of massive splenomegaly and/or thrombocytopenia, in order to avoid potentially harmful splenectomy. This case report describes a 25-year-old female patient with a 10-year medical history of anaemia and thrombocytopenia, who presented with symptoms of haemorrhagic dyscrasia, pancytopenia and massive splenomegaly. The differential diagnosis of massive splenomegaly included several conditions which were considered but ruled out. Because of a lack of resources, the patient was forwarded to a reference centre where the diagnosis of GD was made.
CONCLUSIONS: Many diseases are associated with splenomegaly but massive splenomegaly is seen in only a few conditions.While some causes of splenomegaly are obvious (malaria, infection), the aetiological diagnosis of splenomegaly may involve extensive history taking, serum testing and imaging studies.Infiltrative disorders such as Gaucher disease are a rare cause of splenomegaly and should be considered when other more common causes have been ruled out.The authors hope to raise awareness of this diagnosis in order to encourage early treatment.

Introduction: Massive splenomegaly in children can complicate minimally invasive splenectomy. Splenic artery embolization (SAE) before splenectomy has been shown to decrease splenic volume, reduce intraoperative blood loss, and decrease conversion rates in laparoscopic surgery. Our objective was to review our recent experience with immediate preoperative SAE in massive splenomegaly for pediatric patients using both laparoscopic and robotic techniques. Materials and Methods: We retrospectively reviewed preoperative SAE outcomes in pediatric patients with massive splenomegaly undergoing minimally invasive splenectomy between January 2018 and July 2021. Results: Four patients, 3 female, ages 5-18 years, had SAE immediately before minimally invasive splenectomy. Two cases were completed robotically, one laparoscopically, and one laparoscopic case required conversion to open. SAE time ranged from 69 to 92 minutes. Time between embolization and surgical start ranged from 26 to 56 minutes, with operative times from 153 to 317 minutes. Estimated blood loss ranged from <10 to 150 mL. Mean length of stay was 3.5 days (range 2-6). Postoperative complications included one patient with ileus and another with concurrent gastritis and urinary tract infection. Splenic size comparisons were difficult to perform due to morselization of the spleen; however, excised spleen weights, measurements, and surgeon\'s impression suggested decreased size of the spleen after SAE. There were no transfusions, postembolization complications, or deaths. Conclusion: SAE subjectively appears to decrease splenic distension, which should allow for easier manipulation and possibly better visualization of splenic hilar vessels during minimally invasive surgery. Immediate preoperative SAE is safe and feasible and should be considered in pediatric patients with massive splenomegaly.

OBJECTIVE: The aim of this study was to investigate the efficacy and safety of the combination of low-molecular-weight heparin + dexamethasone after partial splenic embolization in cirrhotic patients with massive splenomegaly.
METHODS: This study included 116 patients with liver cirrhosis complicated with massive splenomegaly who underwent PSE in Union Hospital from January 2016 to December 2019, and they met the criteria. They were divided into two groups: PSE + Hep + Dex group (N = 54) and PSE group (N = 62). We conducted a retrospective study to analyze the efficacy and safety of the two groups of patients.
RESULTS: The volume of splenic embolization was 622.34 ± 157.06 cm3 in the PSE + Hep + DEX group and 587.62 ± 175.33 cm3 in the PSE group (P = 0.306). There was no statistically difference in the embolization rate of the spleen between the two groups (P = 0.573). WBC peaked 1 week after PSE and PLT peaked 1 month after PSE in both groups; it gradually decreased later, but was significantly higher than the preoperative level during the 12-month follow-up period. The incidences of abdominal pain (46.3% vs 66.1%, P = 0.039), fever (38.9% vs 75.8%, P < 0.001), PVT (1.9% vs 12.9%, P = 0.026), refractory ascites (5.6% vs 19.4%, P = 0.027) were lower in the PSE + Hep + DEX group than in the PSE group. The VAS score of abdominal pain in PSE group was higher than that in PSE + Hep + DEX group on postoperative days 2-8 (P < 0.05). Splenic abscess occurred in 1(1.6%) patient in the PSE group and none (0.0%) in the PSE + Hep + DEX group (P = 0.349).
CONCLUSIONS: The combined use of dexamethasone and low-molecular-weight heparin after PSE is a safe and effective treatment strategy that can significantly reduce the incidence of complications after PSE (such as post-embolization syndrome, PVT, refractory ascites).

UNASSIGNED: Malignant lymphoma occurs in all the systemic organs. Rarely, large B-cell lymphoma is located in the spleen, making the diagnosis difficult. Herein, we report a patient presenting with massive splenomegaly due to LBCL. Splenectomy was essential to assess the diagnosis and to guide postoperative therapeutics.
METHODS: A 47-year-old woman, with no comorbidities, complained of weight loss and abdominal pain. She had a palpable spleen that extended below the navel. CT scan revealed massive splenomegaly and lymph nodes in the spleen hilum. Splenectomy was performed. Histopathological examination confirmed the diagnosis of large B-cell lymphoma. The postoperative course was uneventful. Three courses of chemotherapy were given. The patient was in remission after a follow-up of 8 months.
CONCLUSIONS: Massive splenomegaly can be one of the circumstances of the discovery of large B-cell lymphoma. Splenectomy was then essential to confirm the diagnosis and to guide postoperative therapeutics. It also permits reducing hypersplenism and preventing spleen rupture. In patients with high operative risk, splenic needle biopsy should be taken into consideration. Splenic artery embolization before surgery can also be performed in patients having massive splenomegaly to reduce the spleen volume. We highlight the importance of splenectomy to confirm the diagnosis and to relieve the symptoms. Postoperative chemotherapy is essential to prevent relapses.
CONCLUSIONS: Splenectomy is essential in spleen localized large B-cell lymphoma. It permits to confirm the diagnosis, relieve symptoms, and treatment of underlying hematologic malignancies. Postoperative chemotherapy is essential to prevent relapses.

An 85-year-old woman presented with pain and a palpable mass in her left flank. Abdominal computed tomography revealed massive splenomegaly and para-aortic lymphadenopathies. Bone marrow biopsy showed CD79a, CD20, and bcl-2-positive atypical lymphocytes, which led to the diagnosis of splenic marginal zone lymphoma.

Sarcoidosis, a systemic inflammatory disease of unknown etiology, can affect any site in the body. A bone lesion was unexpectedly detected by fluorodeoxyglucose position emission tomography/computed tomography (FDG PET/CT) in a patient with multiorgan sarcoidosis. FDG PET/CT should be considered for the detection of clinically silent lesions of sarcoidosis.

A case demonstrating diagnostic and therapeutic rational for surgical management of massive splenomegaly.