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.

Deficiency of vitamin B12 usually presents with symptoms of anaemia or neurological dysfunction. We report a case of a young lady who was found to be vitamin B12 deficient with massive splenomegaly, haemolytic anaemia, and pancytopenia. She was thoroughly investigated for other causes of similar presentation. Her symptoms and blood count drastically improved after two weeks of therapy with vitamin B12 supplementation. After reviewing the literature on unusual cases of vitamin B12 deficiency, our case is a very interesting read as it serves as a reminder for health care providers to be alert for these manifestations, which can be treated by simply replacing vitamin B12.

Banti\'s syndrome is a chronic congestive enlargement of the spleen leading to the destruction of blood cells resulting in pancytopenia. It is also associated with cirrhosis and ascites along with symptoms of pancytopenia such as infection, bruising, weakness, and fatigue. Multiple factors such as hepatitis B infection, coagulation abnormalities and exposure to arsenic, etc. may also cause Banti\'s syndrome. Clinical evaluation with blood profile along with use of imaging studies such as MRI and splenic venography is utilized for the determination of Banti\'s syndrome. In this report, we present a 29-year-old diabetic male who presented with abdominal distention, right leg cellulitis, fever, and a past history of hematemesis and melena. On examination, distended abdomen showed marked splenomegaly with ascites (positive shifting dullness and fluid thrill). Also, the left leg was warm, swollen, and tender to the touch. Complete blood count showed decreased WBC, RBC, Hb, with peripheral smear negative for malarial parasites. Ultrasound scan of abdomen and pelvis was done illustrating massive splenomegaly with pelvic dilation and 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.

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.

Sarcoidosis is a granulomatous disease with multiple system involvement. It is characterized by the presence of non-caseating granulomas in the involved organs. The most commonly affected organ in sarcoidosis is the pulmonary interstitium. However, extra-pulmonary involvement can be manifested in almost any other organ system. Less commonly, sarcoidosis can manifest with massive splenomegaly. As extensive differential diagnoses, such as hematological malignancies, primary splenic or metastatic tumors, infiltrative disorders, and inflammatory disorders are considered, diagnosing sarcoidosis with the presentation of massive splenomegaly is clinically challenging. Here, we discuss the case of a 56-year-old female with splenic sarcoidosis and skin manifestation.

Hereditary spherocytosis is a rare cause of chronic haemolytic anaemia. It is rarer in the black population with extremely few cases reported. Initial assessment of a patient with suspected disease should include documenting clinical features of chronic haemolysis and a family history. Its management in pregnancy is challenging and needs a multidisciplinary team. We report on a black South African woman with hereditary spherocytosis and massive splenomegaly presenting with severe haemolytic anaemia at 23rd week of pregnancy. She had a successful splenectomy.