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Oncology Letters May 2024Hemophagocytic lymphohistiocytosis (HLH) represents a fatal immunopathology derived from excessive inflammatory reactions. In particular, lymphoma-associated...
Hemophagocytic lymphohistiocytosis (HLH) represents a fatal immunopathology derived from excessive inflammatory reactions. In particular, lymphoma-associated hemophagocytic syndrome (LAHS) is associated with a dismal prognosis. The current study presented a challenging case of splenic LAHS. A 71-year-old man presented with fatigue and anorexia. Laboratory test results revealed anemia, thrombocytopenia, lactate dehydrogenase elevation and markedly elevated levels of ferritin (6,210 ng/ml) and soluble interleukin 2 receptor (sIL-2R; 11,328 U/ml). Abdominal computed tomography revealed marked splenomegaly, while fluorodeoxyglucose positron emission tomography revealed increased tracer uptake in the spleen. An elective splenectomy was performed, which led to the diagnosis of B-cell splenic lymphoma with transformation from indolent to aggressive lymphoma. Prior to the splenectomy, thrombocytopenia and hepatic dysfunction with rapidly progressing jaundice appeared, accompanying further elevation of ferritin (25,197 ng/ml) and sIL-2R levels (30,420 U/ml). On postoperative day 5, the patient was transferred to a tertiary care institution and corticosteroid pulse therapy was immediately initiated after establishing the diagnosis of LAHS. Liver dysfunction gradually recovered and subsequent chemotherapy resulted in complete remission with improved performance status. At eight months after the onset, the patient remains alive without any signs of residual lymphoma. Although splenic lymphoma typically manifests with low-grade lymphoma, it can transform into high-grade lymphoma associated with severe complications, such as HLH and multiple organ failure. In this case, splenectomy assisted in not only establishing the diagnosis but also in tumor cytoreduction before commencing chemotherapy. Through interdisciplinary collaboration, the patient was successfully treated by performing a timely splenectomy, followed by steroid pulse therapy and chemotherapy.
PubMed: 38590310
DOI: 10.3892/ol.2024.14355 -
Medicine Apr 2024Megalosplenia in newly diagnosed multiple myeloma (MM) is extremely rare, posing diagnostic and therapeutic challenges due to its unusual location and clinical... (Review)
Review
INTRODUCTION
Megalosplenia in newly diagnosed multiple myeloma (MM) is extremely rare, posing diagnostic and therapeutic challenges due to its unusual location and clinical manifestations and lack of optimal therapeutic strategies.
CASE PRESENTATION
A 65-year-old female who was previously healthy presented with a history of ecchymosis on her right leg accompanied by progressive fatigue for 2 weeks. She was admitted to our center in July 2019 due to thrombocytopenia. The patient presented with megalosplenia, anemia, monoclonal protein (λ-light chain type) in the serum and urine, and 45.6% malignant plasma cells in the bone marrow. Splenectomy was performed due to persistent splenomegaly after 3 cycles of the bortezomib plus dexamethasone regimen, and immunohistochemistry results indicated λ-plasmacytoma of the spleen. The same cytogenetic and molecular abnormalities, including t(14;16), 14q32 amplification, 16q32 amplification, 20q12 amplification, and a novel CYLD gene mutation, were identified using fluorescence in situ hybridization and next-generation sequencing in both bone marrow and spleen samples. Therefore, a diagnosis of MM (λ-light chain type, DS III, ISS III, R-ISS III, high-risk) with spleen infiltration was proposed. The patient did not achieve remission after induction treatment with bortezomib plus lenalidomide and dexamethasone or salvage therapy with daratumumab plus ixazomib and dexamethasone. However, she ultimately did achieve very good partial remission with a regimen of bendamustine plus lenalidomide and dexamethasone. Unfortunately, she died of pneumonia associated with chemotherapy.
CONCLUSION
To our knowledge, only 8 cases of spleen plasmacytoma at MM diagnosis have been described previously. Extramedullary myeloma patients with spleen involvement at diagnosis are younger and that the condition is usually accompanied by splenic rupture with aggressive clinical features and poor prognosis. Further studies are needed to explore pathogenesis and effective therapies to prolong the survival of such patients.
Topics: Humans; Female; Aged; Multiple Myeloma; Lenalidomide; Bortezomib; Plasmacytoma; In Situ Hybridization, Fluorescence; Dexamethasone; Mutation; Antineoplastic Combined Chemotherapy Protocols; Deubiquitinating Enzyme CYLD
PubMed: 38579060
DOI: 10.1097/MD.0000000000037624 -
Medicine Apr 2024Primary myelofibrosis is a subtype of myeloproliferative neoplasm that leads to bone marrow fibrosis. Historically, the only curative option for primary myelofibrosis... (Review)
Review
RATIONALE
Primary myelofibrosis is a subtype of myeloproliferative neoplasm that leads to bone marrow fibrosis. Historically, the only curative option for primary myelofibrosis was allogeneic hematopoietic stem cell transplant. Ruxolitinib, a Janus kinase inhibitor, is now used for the treatment of primary myelofibrosis and polycythemia vera. It effectively improves symptoms related to splenomegaly and anemia. However, its association with the development of opportunistic infections has been observed in clinical studies and practical application.
PATIENT CONCERNS
A 64-year-old female with primary myelofibrosis and chronic hepatitis B infection who received ruxolitinib treatment. She was admitted for spiking fever and altered consciousness.
DIAGNOSIS
Tuberculosis meningitis was suspected but cerebrospinal fluid can't identify any pathogens. An abdominal computed tomography scan revealed a left psoas abscess and an enlarged spleen. A computed tomography-guided pus drainage procedure was performed, showing a strong positive acid-fast stain and a positive Mycobacterium tuberculosis polymerase chain reaction result.
INTERVENTIONS
antituberculosis medications were administered. The patient developed a psoas muscle abscess caused by tuberculosis and multiple dermatomes of herpes zoster during antituberculosis treatment.
OUTCOMES
The patient was ultimately discharged after 6 weeks of treatment without apparent neurological sequelae.
LESSONS
This case underscores the importance of clinicians evaluating latent infections and ensuring full vaccination prior to initiating ruxolitinib-related treatment for primary myelofibrosis.
Topics: Female; Humans; Middle Aged; Nitriles; Primary Myelofibrosis; Psoas Abscess; Psoas Muscles; Pyrazoles; Pyrimidines; Splenomegaly; Tuberculosis
PubMed: 38579059
DOI: 10.1097/MD.0000000000037653 -
Annals of Medicine and Surgery (2012) Apr 2024Hydatosis is a zoonotic parasitic disease caused by echinococcosis larval infection. South America, Africa, the Middle East, South Europe, India, and Australia are...
INTRODUCTION
Hydatosis is a zoonotic parasitic disease caused by echinococcosis larval infection. South America, Africa, the Middle East, South Europe, India, and Australia are endemic to this disease. Splenic involvement is a rare and complicated hydatid disease presentation. A splenic hydatid cyst is an infrequent clinical occurrence, even in regions where the disease is endemic.
CASE PRESENTATION
A 16-year-old male, having a background of mild abdominal trauma and non-resolving dull abdominal pain attended a paediatric surgical outpatient office and following a thorough examination, was diagnosed with a giant solitary isolated splenic hydatid cyst. Subsequently, the patient received albendazole and underwent total splenectomy, necessitated by the considerable size of the cyst, classified as a giant.
CLINICAL DISCUSSION
Splenic involvement of hydatid disease is a rare presentation (0.5-8%.). With the initial clinical finding often involving the accidental discovery of a palpable mass, the most frequently reported symptoms and signs include the presence of a palpable mass, fever, dull pain, or splenomegaly. Ultrasound and computed tomography are the most helpful tools for evaluating focal splenic diseases. The preferred treatment involves the use of antihelminthic drugs such as albendazole or mebendazole in conjunction with splenectomy. Total splenectomy is the preferred approach and is associated with decreased hospital stay, reduced healthcare costs, and a lower likelihood of recurrence.
CONCLUSION
in endemic areas, in patients with splenic cysts, hydatidosis should be contemplated.
PubMed: 38576966
DOI: 10.1097/MS9.0000000000001851 -
Cureus Mar 2024Background Coronavirus disease 2019 (COVID-19), resulting from the severe acute respiratory syndrome corona virus 2 (SARS-CoV-2), has not only shown substantial effects...
Background Coronavirus disease 2019 (COVID-19), resulting from the severe acute respiratory syndrome corona virus 2 (SARS-CoV-2), has not only shown substantial effects on the respiratory system but also on extrapulmonary systems, including cardiovascular, gastrointestinal, hematological, and immune responses, notably spleen enlargement. The connection between the enlargement of the spleen and pulmonary complications in individuals with COVID-19 is still not well elucidated, with current studies offering divergent conclusions. Objective This study aims to elucidate the correlation between splenomegaly, as assessed by computed tomography (CT) imaging, and the extent of lung involvement (LI) in COVID-19 patients, thereby offering insights into potential prognostic indicators. Methodology A hospital-based, cross-sectional, retrospective study was conducted involving 1058 symptomatic COVID-19 patients confirmed by reverse transcriptase-polymerase chain reaction (RT-PCR), aged 18 years and above. CT imaging was utilized to evaluate spleen size and LI. Statistical analyses, including Pearson correlation and simple linear regression, were performed to explore the relationship between spleen size and LI. Results The study cohort exhibited a mean spleen size of 9.49 cm and a mean LI score of 0.272. The Pearson correlation coefficient was calculated at 0.0495, indicating a marginal positive correlation between spleen size and LI. Regression analysis demonstrated a minimal impact of spleen size on LI, with spleen size accounting for only 0.2% of the variance in LI scores. Conclusions The study found a slight, statistically non-significant correlation between splenomegaly and LI in COVID-19 patients, suggesting that while splenic enlargement may reflect systemic disease involvement, it is not a strong independent predictor of lung damage extent. The findings highlight the complexity of extrapulmonary manifestations and highlight the need for additional research to fully understand the implications of splenic involvement in COVID-19.
PubMed: 38567206
DOI: 10.7759/cureus.55415 -
Cureus Mar 2024Splenic marginal zone lymphoma (SMZL) usually presents with splenomegaly or symptoms related to cytopenia. We report a case of a 56-year-old female with previously...
Splenic marginal zone lymphoma (SMZL) usually presents with splenomegaly or symptoms related to cytopenia. We report a case of a 56-year-old female with previously diagnosed antiphospholipid syndrome (APS) on warfarin therapy who initially presented with abdominal pain and was found to have massive splenomegaly and splenic infarction on CT imaging. Initial clinical presentations and imaging findings were attributed to the subtherapeutic coagulation profile. The patient was later diagnosed with SMZL following workup for pancytopenia including bone marrow biopsy, flow cytometry, and PET scan. Cytopenias, splenomegaly, and abnormal metabolic activity in the spleen on the PET scan improved after treatment with four cycles of weekly rituximab. Our report presents a case of a patient with longstanding APS presenting with splenic infarction and pancytopenia who was subsequently diagnosed with SMZL and successfully treated with rituximab.
PubMed: 38562339
DOI: 10.7759/cureus.55374 -
Acta Medica Indonesiana Jan 2024A 44-year-old female presented with a distended abdomen and fatigue. On physical examination, prominent splenomegaly was found. The laboratory investigations revealed...
A 44-year-old female presented with a distended abdomen and fatigue. On physical examination, prominent splenomegaly was found. The laboratory investigations revealed pancytopenia and decreased albumin-globulin ratio. The abdominal ultrasonography revealed splenomegaly, cholelithiasis, and cystitis, and the bone survey showed osteopenia. Differential diagnoses included leukemia, multiple myeloma, and myelofibrosis therefore bone marrow puncture was performed. However, histopathologic examination found Gaucher-like cells in the bone marrow aspiration. The finding of CD68 positivity in Gaucher-like cells by using the immunohistochemistry staining supporting Gaucher disease. To confirm the diagnosis, an examination of glucocerebroside substrate from the patient's blood plasma was performed. Glucosylsphingosine, a deacylated form of glucosylceramide, was markedly elevated. Therefore, the diagnosis of Gaucher disease was confirmed. This is the first reported adult Gaucher case diagnosed in Indonesia.
Topics: Adult; Female; Humans; Gaucher Disease; Splenomegaly; Indonesia; Pancytopenia; Diagnosis, Differential
PubMed: 38561877
DOI: No ID Found -
Heliyon Apr 2024, is a potential functional strain with the capacity to regulate intestinal health and ameliorate colonic inflammation. However, the strain requires further...
, is a potential functional strain with the capacity to regulate intestinal health and ameliorate colonic inflammation. However, the strain requires further investigation regarding its safety profile and potential mechanisms of colitis improvement. In this study, the safety of 98D (Ed) as a potential probiotic was studied using methods. Additionally, a dextran sulfate sodium (DSS)-induced murine colitis model was employed to investigate its impact on the intestinal microbiota and colitis. antimicrobial assays revealed Ed sensitivity to common antibiotics and its inhibitory effect on the growth of O157, CCUG 37328, and ATCC 25923. To elucidate the functional properties of Ed, 24 weight-matched 6-week-old female C57BL/6J mice were randomly divided into three groups (n = 8): NC group, Con group (DSS), and Ed group (DSS + Ed). Ed administration demonstrated a protective effect on colitis mice, as evidenced by improvements in body weight, colonic length, reduced disease activity index, histological scores, diminished splenomegaly, and decreased goblet cell loss. Furthermore, Ed downregulated the expression of the pro-inflammatory cytokine genes (, , and ) and upregulated the expression of the anti-inflammatory cytokine gene . The 16S rRNA gene sequencing revealed significant alterations in microbial α-diversity, with principal coordinate analysis indicating distinct differences in microbial composition among the three groups. At the phylum level, the relative abundance of Actinomycetota significantly increased in the Ed-treated group. At the genus level, Ed treatment markedly elevated the relative abundance of , , and in DSS-induced colitis mice. In conclusion, Ed exhibits potential as a safe and effective therapeutic agent for DSS-induced colitis by reshaping the colonic microbiota.
PubMed: 38560132
DOI: 10.1016/j.heliyon.2024.e28486 -
The Journal of Veterinary Medical... May 2024A 7-year-and-8-month-old, male degu (Octodon degus) with anorexia, depression, and labored breathing was found to have a thoracic effusion and enlargement of the right...
A 7-year-and-8-month-old, male degu (Octodon degus) with anorexia, depression, and labored breathing was found to have a thoracic effusion and enlargement of the right testis on radiographic examination. Despite treatment, the animal died. At necropsy, hepatomegaly, splenomegaly, and multifocal nodules on the intestinal serosa and mesentery were observed. Histologically, the foci were densely cellular invasive neoplasms composed of sheets of round to polygonal cells, with occasional multinucleated giant cells. Immunohistochemically, the neoplastic cells were immunopositive for ionized calcium-binding adapter molecule 1, human leukocyte antigen-DR, and CD204. These findings were consistent with disseminated histiocytic sarcoma.
Topics: Animals; Histiocytic Sarcoma; Male; Octodon; Fatal Outcome
PubMed: 38556322
DOI: 10.1292/jvms.24-0081 -
Viruses Mar 2024Sturgeon farming is rapidly expanding in Europe, where Italy ranks first in farmed caviar production. A major threat to sturgeon health in captivity is infection with...
Sturgeon farming is rapidly expanding in Europe, where Italy ranks first in farmed caviar production. A major threat to sturgeon health in captivity is infection with Acipenser European Iridovirus (AcIV-E), a viral disease definitively identified in 2016. Here we present data on the occurrence of AcIV-E in 482 sturgeons (age ≤ 12 months, species of the genus and the species ) collected from sturgeon farms in northern Italy between January 2021 and December 2023. The health status of each specimen was determined by necroscopy and virological assay. Virological analysis was performed on gill samples and real-time PCR specific to the MCP gene of the iridovirus viral capsid. Molecular analysis revealed positivity to the virus in 204 samples (42.68% of the total), while anatomopathological examination of nearly all fish with positive real-time PCR disclosed swollen abdomen, hepatic steatosis, splenomegaly, and increased gill volume. Two challenges to timely diagnosis are the absence of pathognomonic symptoms and the inability to isolate the virus on cell monolayers. Continuous and widespread health monitoring is therefore crucial for disease management and to effectively control spread of the virus.
Topics: Animals; Iridovirus; Fish Diseases; Italy; Virus Diseases; Europe; Fishes
PubMed: 38543830
DOI: 10.3390/v16030465