Splenic cyst deroofing complicated with B lymphoma.

BACKGROUND: Splenic cysts are uncommon and very rarely malignant therefore their treatment isn't standardized. In case of symptomatic cysts different surgical approaches have been suggested. Primary malignant lymphoma of the spleen comprises less than 1% of non-Hodgkin's lymphomas. To our knowledge, only 203 cases of splenic large B-cell lymphoma (LBCL) have been reported to date and only 2 of them were fibrin-associated splenic cysts. CASE PRESENTATION: 27-year-old model with a 19 × 13 cm splenic cyst without data of malignancy in the preliminary study and therefore treated with laparoscopic deroofing. After histological diagnosis of LBCL with a fibrin/EBV-associated splenic pseudocyst, the patient received 4 cycles of Rituximab and a laparoscopic splenectomy was performed due to resurgence of the pseudocyst. No evidence of malignancy has been found during follow up (EBV viral load every 3 months during the first year, PET-CT every 6 months during the first year and annual afterwards) performed after the splenectomy. DISCUSSION AND CONCLUSIONS: The value of tumor markers and radiology for diagnosis of splenic cysts is put into question. Only 60 cases of Fibrin-associated LBCL (FA-LBCL) have been described in the literature therefore there are no treatment guidelines for them even though surgery together with systemic treatment has been the prevalent route with good results in most cases.

[Genetic analysis of a child with Hereditary hemorrhagic telangiectasia type I in conjunct with Splenic sinus shore cell hemangioma].

OBJECTIVE: To explore the genetic basis and pathogenesis for a child with type I Hereditary hemorrhagic telangiectasia (HHTⅠ) and Splenic sinus shore cell hemangioma (LCA). METHODS: A child with HHT complicated with LCA diagnosed at the First Affiliated Hospital of Dali University in April 2022 was selected as the study subject. Clinical data of the child and her relatives were collected, and pathogenic variants were screened by whole exome sequencing. Candidate variant was verified by Sanger sequencing and bioinformatic analysis. RESULTS: The patient, a 16-year-old female, had recurrent epitaxis since childhood, which sometimes necessitated hemostasis treatment. She also had splenectomy due to splenic rupture and was diagnosed with LCA. Her father and grandmother also had a history of recurrent epitaxis. Her father had deceased due to cerebral vascular rupture. The child was found to harbor a c.360+1G>A variant in the ENG gene. The same variant was not found in her asymptomatic mother and brother. CONCLUSION: The c.360+1G>A variant of the ENG gene probably underlay the pathogenesis in this child.

Paws and Pathogens: A Multidisciplinary Approach Through Bartonella henselae Endocarditis and Hepatosplenic T-Cell Lymphoma.

Bartonella species are gram-negative coccobacilli that are globally recognized as significant pathogens causing zoonotic infections. Among Bartonella species, B. henselae, B. quintana, and B. bacilliformis are prominent pathogens causing infections in humans, often manifesting as infective endocarditis. Bartonella endocarditis can pose diagnostic challenges due to its indolent presentation and limitations of standard microbiological culture techniques to identify the organism. We report a case of a 23-year-old male, who initially presented with the manifestations of hepatosplenic T-cell lymphoma, later diagnosed with blood culture-negative endocarditis caused by B. henselae. The patient had a complicated clinical course including pancytopenia, hepatosplenomegaly, and severe aortic valve regurgitation. Despite negative blood cultures, diagnostic clues included persistent fevers and bicuspid aortic valve with abscess. High Bartonella IgG titers (>1:800) supported the diagnosis. This case underscores the importance of considering Bartonella species in patients with suspected endocarditis, particularly in those with predisposing factors and negative blood cultures. Diagnosis relies heavily on serologic assays due to low sensitivity of conventional culture methods. Treatment involves a multidisciplinary approach with antibiotics and surgical intervention for optimal outcomes. Timely recognition and management are crucial to mitigate the high mortality associated with Bartonella endocarditis, and we hope this article offers insight for clinicians.

A case of multifocal sclerosis angiomatoid nodular transformations of the spleen occurring after partial splenic infarction with transcatheter arterial embolization for splenic artery aneurysm.

A 48-year-old woman underwent transcatheter arterial embolization (TAE) for a splenic artery aneurysm, which resulted in a partial splenic infarction in the middle lobe. Five years after TAE, a 20-mm diameter mass in the noninfarcted area of the spleen was detected on imaging, which grew to 25 mm in diameter after 6 months. MRI after gadolinium administration showed a 35 × 34 mm mass within the superior pole and 15 × 12 mm mass within the inferior pole. The patient underwent laparoscopic splenectomy and had an uneventful postoperative recovery. No evidence of recurrence was observed during the 2-year follow-up period after surgery. The mass was pathologically confirmed to be sclerosing angiomatoid nodular transformation (SANT) of the spleen. While some studies hypothesize that SANT is a response to vascular injury or trauma, to the best of our knowledge, there have been no previous reports of SANT occurring after procedures directly affecting splenic blood flow. Additionally, multifocal SANTs are reported to be very rare, accounting for only 4.7% of all reported SANTs of the spleen. We highlight a rare course of SANT of the spleen and discuss the possible relationship between blood flow abnormalities and the appearance of SANT.

Hepatosplenic T-cell lymphoma presented with massive splenomegaly and pancytopenia - a case report.

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. CASE: 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. CONCLUSION: 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.

Littoral cell angioma discovered after splenectomy in a patient with non-Hodgkin lymphoma and melanoma. Case report and review.

BACKGROUND: Littoral cell angioma (LCA) is a rare vascular primary tumor of the spleen with no more than 440 cases described so far. Although often seen as benign, it is described to have malignant potential and to be associated with other immunologic disorders or malignancies. CASE PRESENTATION: We present the case of LCA in a 75-year old man with a concomitant non-Hodgkin lymphoma and medical history of malign melanoma. The tumor was discovered incidentally after splenectomy for splenomegaly and refractory thrombocytopenia. The post-operative period was uneventful. CONCLUSION: Our case is the first to report an association of LCA with both lymphoma and melanoma thus far. It emphasizes the need for a thorough total body examination for synchronous diseases and close follow-up to reveal associated malignancies or immunologic disorders. Further research is required to identify etiologic and pathogenetic mechanisms behind this tumor and a common basis between the three diseases. KEY WORDS: Littoral Cell Angioma, Neoplasm, Splenectomy, Solid Spleen Tumor.

Non-Small Cell Lung Cancer Causing Atraumatic Splenic Rupture Without Splenic Metastasis.

Atraumatic splenic rupture (ASR) is a rare occurrence but an important clinical entity. Although trauma is the most common cause of splenic rupture, there is limited literature on ASR. This case report discusses a 59-year-old woman presenting with tension hydrothorax and ASR in the setting of non-small cell lung carcinoma requiring emergent chest tube insertion and emergent splenectomy. Her hospital course was complicated by pulmonary embolism and thrombosis of the inferior vena cava. The patient expired three months after her initial presentation. This patient's presentation represents only the second documented case of atraumatic splenic rupture secondary to metastatic lung carcinoma without pathological evidence of splenic metastasis. Atraumatic splenic rupture secondary to metastatic NSCLC is a rare occurrence; though failure to detect, it may be fatal. Pathologic ASR may be an occult presentation of lung malignancy and in the presence of confirmed NSCLC may portend a poor prognosis.

[Littoral cell angioma]. / Dongasejtes angioma.

Splenic rupture secondary to blunt trauma is a common condition. Non-traumatic, also known as spontaneous or pathological splenic rupture is an uncommon, but potentially life-threatening condition. Spontaneous splenic rupture caused by a primary splenic tumor is rare. In this case study, we present a special, benign tumor causing splenic rupture. Our 78-year-old female patient was hospitalized due to left shoulder pain and chest discomfort. Her blood pressure was low, the laboratory tests showed anemia, and the chest CT scan involving also the upper abdomen raised the suspicion of a splenic rupture. During the emergency splenectomy, there was a large amount of blood in the abdominal cavity. Macroscopic pathological examination of the removed spleen showed multifocal cystic lesions that led to splenic rupture. Immunhistochemical analyses revealed a littoral cell angioma. Littoral cell angioma is a rare, benign vascular tumor of the spleen, which is thought to originate from the red pulp sinuses lined with littoral cells. The aim of our report is to describe an unusual cause of sudden splenic rupture without traumatic history, the histologically benign littoral cell angioma that has not been published in Hungary. Orv Hetil. 2023; 164(10): 393-397.

Splenic surprise in a case of renal cell carcinoma: Unusual case or association?

Littoral cell angioma (LCA) is a rare benign tumor originating exclusively from the venous sinus lining cells of the splenic red pulp. These cells are unique in having a distinctive hybrid endothelial/histiocytic phenotype. Also, there are reports of the association of LCA with internal malignancies. We present a case report highlighting an unusual association of LCA with conventional renal cell carcinoma (RCC), masquerading as a metastatic lesion. Knowledge of such an association is necessary to avoid misdiagnosis and prevent potential overtreatment.

Prolonged fever with pancytopenia and massive hepatosplenomegaly.

Prolonged fever with pancytopenia and hepatosplenomegaly is a clinical entity frequently encountered by physicians. The diagnosis of such cases is challenging due to the diversity of differential diagnoses. Hepatosplenic T-cell lymphoma is a rare and aggressive type of non-Hodgkin lymphoma that can present with massive hepatosplenomegaly, pancytopenia and prolonged fever. Most of the patients are young men and the majority are associated with chronic immunosuppression. We report a 40-year-old immunocompetent woman with prolonged fever and pancytopenia due to hepatosplenic T-cell lymphoma.

[Metastatic lesion of the bone marrow caused by primary angiosarcoma of the spleen]. / Metastaticheskoe porazhenie kostnogo mozga pri pervichnoi angiosarkome selezenki.

The article presents a rare case of bone marrow metastasis of the spleen angiosarcoma. The observation is of particular interest due to the fact that secondary bone marrow damage in angiosarcoma in the vast majority of cases is due by primary tumor growth in the spleen. Clinically, such cases may resemble the course of blood diseases with hematological disorders and splenomegaly. Patients come into the field of view of a hematologist, and the final diagnosis is unexpected to the attending physician. Detection of angiosarcoma growth in a bone marrow trephine biopsy during morphological examination can be a rare finding for a pathologist. In this regard, the presented case is of interest not only for pathologists, but also for doctors of clinical specialties.

Evaluation of the validity of the double two-thirds rule for diagnosing hemangiosarcoma in dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass: a systematic review.

OBJECTIVE: To evaluate the validity of the double two-thirds rule for a diagnosis of splenic hemangiosarcoma in dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass. SAMPLE: Systematic literature review. PROCEDURES: 3 databases (PubMed, CAB abstracts, and World of Science) were searched in November 2020. Articles were included if data on dogs with nontraumatic hemoperitoneum due to a splenic mass were included and subsequent pathologic diagnosis could be determined. RESULTS: In total, 2,390 unique articles were identified, with 66 articles meeting the criteria for full-text review and 14 articles included for analysis. A total of 1,150 dogs were evaluated, with 73.0% (840/1,150) of dogs being diagnosed with a malignant splenic lesion and 27.0% (310/1,150) being diagnosed with a benign splenic lesion. Of the malignancies, 87.3% (733/840) were hemangiosarcoma. Levels of evidence were low, and bias was high as most included studies were retrospective case series. CLINICAL RELEVANCE: The double two-thirds rule should be refined when evaluating dogs with nontraumatic hemoperitoneum from a ruptured splenic mass, with more dogs being diagnosed with a malignancy and hemangiosarcoma specifically than the double two-thirds rule indicates. These findings may be useful in an emergency setting to guide owners on potential diagnoses for dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass. However, there remains a portion of these dogs with benign conditions and nonhemangiosarcoma malignancies that may have a good long-term prognosis compared to dogs with hemangiosarcoma. Studies with higher levels of evidence, lower risks of bias, and large case numbers are needed in the literature.

[Warm autoimmune hemolytic anemia and IgM-monoclonal gammopathy following BNT162b2 COVID-19 vaccine in a patient with splenic marginal zone lymphoma].

There is currently no evidence that a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mRNA vaccine might be associated with the development of autoimmune hemolytic anemia or disease progression in patients with mature B-cell neoplasm. Our patient was a 71-year-old man with indolent mature B-cell neoplasm who had been monitored for many years without treatment. After receiving the second dose of the BNT162b2 mRNA COVID-19 vaccine, he developed severe warm autoimmune hemolytic anemia. Although steroid therapy improved his anemia, he continued to develop IgM-monoclonal gammopathy, renal insufficiency, and splenomegaly. He was diagnosed with splenic marginal zone lymphoma after undergoing splenectomy. The splenectomy improved the patient's symptoms. We assessed his SARS-CoV-2 specific antibody response, but the patient's serologic response to the vaccine was impaired. In patients with mature B-cell neoplasm, a non-specific immune response after vaccination might be associated with paraneoplastic syndromes.

Capillaritis as a Skin Manifestation of Hepatosplenic Gamma-Delta T-Cell Lymphoma.

We present the case of a 10-year-old boy who was admitted with 3 months of episodic febrile neutropenia and a new petechial rash. Routine bloods identified neutropenia, thrombocytopenia, and a raised alanine aminotransferase. The dermatology team was consulted in light of the symmetrical petechial eruption of the upper torso. A punch biopsy of the lesion was consistent with early capillaritis. The results showed superficial dermal red blood cell extravasation with mild perivascular lymphohistiocytic inflammatory infiltrate. There was no evidence of an atypical lymphoid infiltrate in the skin biopsy. An initial bone marrow aspirate showed an abnormal mature T-cell population consisting of CD4 and CD8 T cells with gamma-delta positivity. Karyotyping was also done, which demonstrated isochromosome 7q. These findings were consistent with a diagnosis of hepatosplenic T-cell lymphoma (HSTL). The patient underwent fourth-line chemotherapy due to refractory relapsing disease but sadly passed away within 12 months of diagnosis. HSTL is a rare and aggressive subset of peripheral T-cell lymphoma. Prognosis is poor with a median survival of <1 year from diagnosis. However, reports suggest improved outcomes if intensive, early, high-dose chemotherapy is used alongside hematopoietic stem cell transplantation. Therefore, there is an impetus to attain early diagnosis for aggressive early treatment and improved patient outcomes. Capillaritis, presenting as asymptomatic nonpalpable purpura, can be a rare presenting feature of HSTL. Dermatologists could play a pivotal role in the early recognition of this rare but aggressive hematological malignancy and promote prompt treatment resulting in better patient outcomes.

Premature death in dogs with nontraumatic hemoabdomen and splenectomy with benign histopathologic findings.

OBJECTIVE: To determine whether premature death occurred among dogs with nonmalignant splenic histopathologic findings after splenectomy for nontraumatic hemoabdomen. ANIMALS: 197 dogs with nontraumatic hemoabdomen that underwent splenectomy and histopathologic evaluation between 2005 and 2018. PROCEDURES: Information was obtained from electronic medical records, dog owners, and referring veterinarians to determine patient characteristics, histopathologic findings, survival information, and cause of death. Dogs were grouped based on histopathological diagnosis and outcome, and median survival times (MSTs) and risk factors for death were determined. RESULTS: Histopathologic findings indicated malignancy in 144 of the 197 (73.1%) dogs with nontraumatic hemoabdomen. Hemangiosarcoma was diagnosed in 126 dogs (87.5% of those with malignancies and 64.0% of all dogs). Nine of 53 (17%) dogs with nonmalignant histopathologic findings had an adverse outcome and premature death, with an MST of 49 days. Risk factors for this outcome included low plasma total solids concentration, an elevated hemangiosarcoma likelihood prediction score, and a medium or high hemangiosarcoma likelihood prediction score category. CONCLUSIONS AND CLINICAL RELEVANCE: This study showed that there is a group of dogs with nontraumatic hemoabdomen due to splenic disease that have nonmalignant histopathologic findings after splenectomy, but nonetheless suffer an adverse outcome and die prematurely of a suspected malignancy. Further evaluation of potential at-risk populations may yield detection of otherwise overlooked malignancies.

Sclerosing Angiomatoid Nodular Transformation (SANT): A Rare Splenic Tumor and Unusual Cause of Anemia.

BACKGROUND Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign disease of the splenic red pulp of unknown etiology. Definite diagnosis is made on histopathology alone as it has no diagnostic radiologic characteristics. CASE REPORT We present a case of a large incidental splenic mass in a middle-aged man, whose refractory anemia resolved with splenectomy. Our initial imaging studies failed to differentiate this lesion from other splenic lesions like hamartoma and haemangioma. To the best of our knowledge, no SANT cases in the literature have been investigated with red cell scan, as performed in our patient, nor has any case had resolved anemia after treatment. Splenectomy was offered as malignancy could not be safely excluded. Histopathology confirmed the diagnosis of SANT. The patient made good recovery and had no signs of recurrence 2 years later. CONCLUSIONS SANT of the spleen is a rare condition that can mimic many other splenic tumors and poses diagnostic challenge when histopathology is unavailable. Our case adds to the number of SANT reported in literature in the hope of elucidating the pathophysiology of this rare condition.