Splenic cysts are uncommon and are classified as primary cysts or secondary cysts based on the presence or absence of an intact epithelial lining, respectively. Epidermoid cysts have an epithelial lining of stratified squamous epithelium and have been reported in normal and intra-pancreatic accessory spleens. An 18-year-old girl presented with a symptomatic, uncomplicated splenic cyst causing massive splenomegaly and symptoms due to mass effect. Peripheral cytopaenias and lymphoproliferative neoplasm were excluded. Preoperative computed tomography was suggestive of a large splenic cyst causing massive splenomegaly and marked loco-regional pressure effects. Open splenectomy was performed and histology confirmed an epidermoid cyst with no features of malignancy. Splenic epidermoid cysts are a rare cause of massive splenomegaly. Surgical intervention is recommended due to symptoms and the risk of complications.

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.

Hairy cell leukemia variant (HCLv) is a sporadic, B-cell non-Hodgkin lymphoma classified under chronic lymphoproliferative disorders. HCLv usually presents with easy fatigue, dragging pain abdomen, anemia, splenomegaly, hepatomegaly, initially leukocytosis followed by leucopenia, hairy cells in the smear and bone marrow, and an increased risk of infections. There is hypercellular bone marrow, and cytopenias are secondary to hypersplenism. It is essential to differentiate HCL from disorders like classic hairy cell leukemia (HCLc), splenic marginal zone lymphoma, and splenic diffuse red pulp lymphoma, as these are biologically different, with divergent approaches and outcomes. HCLv is poorly responsive or primary refractory to standard purine analogs cladribine or pentostatin. It has lower response rates to even cladribine and rituximab combination, a standard of care for classic HCL with very good response rates. Here, we present a case of an elderly male who presented with splenomegaly and leukocytosis, diagnosed as HCLv, and was treated with a cladribine and rituximab-based regime but showed residual cells in bone marrow on flow cytometry at six months post-treatment. There were no residual cells in peripheral blood in flow cytometry. Various aspects of the disease are discussed here with a detailed literature analysis. There is a definite unmet need for research on better treatment options in HCLv to improve its overall outcome.

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.

Hairy cell leukemia (HCL) is an infrequently encountered chronic B-lymphocyte hematological malignancy, which is found to be more prevalent in males. HCL can present with a myriad of nonspecific symptoms involving the reticuloendothelial system. Usually, patients are diagnosed after an incidental finding of pancytopenia. In the majority of cases, HCL follows an indolent course, and many patients remain asymptomatic. Treatment with nucleoside analogs is the first line of treatment and is indicated for patients with severe anemia, thrombocytopenia, neutropenia, or severe systemic symptoms. Here, we report an atypical case of a 41-year-old Hispanic female who presented with menorrhagia and iron deficiency anemia. She was diagnosed with HCL after a bone marrow biopsy demonstrated the characteristic \"hairy projections.\"

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.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening systemic inflammatory disease. Multiple risk factors have been defined for the manifestation of HLH. While infection remains the top risk factor, having multiple surgical procedures has also been suggested as a potential risk factor for HLH. Our patient presented with generalized weakness, weight loss, and fatigue after having a greenlight laser prostatectomy for benign prostate hypertrophy; the patient deteriorated rapidly and was found to fulfill the HLH 2004 and modified criteria. We believe this patient had a rare bone marrow disorder with a rare complicated clinical and laboratory presentation.

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).