Impact of trastuzumab emtansine (T-DM1) on spleen volume in patients with HER2-positive metastatic breast cancer.

BACKGROUND: Trastuzumab emtansine (T-DM1) is a novel therapy for human epidermal growth factor receptor 2 (HER2)-positive metastatic breast cancer, combining the targeted action of trastuzumab with the cytotoxic effects of emtansine. Although T-DM1 has demonstrated greater efficacy and safety compared to traditional therapies, concerns about hepatotoxicity and spleen-related complications have arisen. METHODS: We conducted a retrospective study of 64 HER2-positive metastatic breast cancer patients treated with T-DM1 at our institution. Patients underwent computed tomography or magnetic resonance imaging at baseline and during treatment cycles. Spleen volume, portal vein diameter, and laboratory values were compared between baseline and 12 months after T-DM1 treatment. RESULTS: Median spleen volume significantly increased from 201 cm3 (IQR, 157-275) at baseline to 291 cm3 (IQR, 215-420) after 12 months of T-DM1 treatment (P < 0.001). Spleen enlargement was observed in 87.5% of patients, while no significant alteration was detected in portal vein diameter. The change in spleen volume was positively correlated with changes in serum globulin levels, liver enzymes, and bilirubin levels, but did not impact survival outcomes. CONCLUSIONS: T-DM1 therapy in HER2-positive metastatic breast cancer leads to significant spleen enlargement and systemic biochemical changes. Future studies should focus on elucidating the long-term implications of these findings and optimizing monitoring strategies for spleen-related complications.

Disseminated Melioidosis Complicated by Prostatic Abscess and Splenic Involvement: Diagnostic and Therapeutic Insights.

This case report details the clinical course of a 53-year-old male farmer with a 15-year history of diabetes mellitus who presented with a 20-day history of pyrexia, rigors, and shivering, as well as problems in the urogenital system and left hypochondrial pain. Notably, he had been diagnosed with spinal tuberculosis, which was successfully treated five years ago. On evaluation, there was tenderness in the suprapubic region as well as the left hypochondrium; moreover, rectal examination showed that the prostate was boggy and tender. The laboratory tests revealed microcytic hypochromic anemia, increased inflammatory markers, and uncontrolled diabetes. Imaging studies reported splenomegaly containing multiple low-density lesions accompanied by cystitis and a prostatic abscess. Positive blood culture samples indicated Burkholderia pseudomallei, thereby signifying disseminated melioidosis. He underwent cystoscopy followed by prostatic deroofing and received intravenous meropenem and prolonged oral cotrimoxazole treatment thereafter. Within 10 days after the initiation of treatment, significant symptomatic relief was achieved. This report highlights the need for a high index of suspicion for melioidosis in systemic infection patients with diabetes and emphasizes prompt surgical intervention along with appropriate medical therapy in complex cases, especially those involving a non-clear-cut diagnosis or severe disease presentation.

A Diagnostic Dilemma: A Case of Angiosarcoma Presenting as Splenomegaly and Pathologic Fracture.

BACKGROUND: Angiosarcomas are rare tumors that can be difficult to diagnose due to subtle changes in the vascular endothelium. When there is evidence to suggest malignancy, such as a pathologic fracture, further investigation is needed, and a high suspicion for angiosarcoma needs to be present so that appropriate immunohistochemical stains are utilized on biopsied tissue. In situations where such suspicion is high and prior biopsies have been negative, performance of splenectomy, can be both diagnostic and therapeutic when splenomegaly is present. CASE REPORT: This is a case of a 52-year-old woman with splenomegaly, initially attributed to infection, in the setting of upper respiratory symptoms and thrombocytopenia. Three months later, however, she presented with back pain. Imaging showed lytic bone lesions with pathologic vertebral fracture and numerous liver lesions that were too small to characterize further. Initial biopsies of the liver and bone did not reveal a pathologic process. Several months later, still without a unifying diagnosis, she presented to our institution. MRI of the brain was done for neurologic concerns and showed pathologic enhancement in the calvarium. A PET scan showed diffuse avidity of the skeleton and spleen. After discussing the case with a hematologist, splenectomy was performed for both diagnostic and therapeutic purposes. Angiosarcoma was identified in the spleen and in a PET-directed bone biopsy. With a definitive diagnosis, she returned home and subsequently elected to pursue hospice care. CONCLUSION: When there is a high clinical suspicion for malignant angiosarcoma, a multidisciplinary approach is necessary to direct both tissue acquisition and necessary histochemical staining.

Liver Function Test Results Correlate With Spleen Size in Patients With Infectious Mononucleosis.

INTRODUCTION: We aimed to evaluate the efficacy of measuring transaminase levels to determine the resolution of splenomegaly in athletes diagnosed with infectious mononucleosis (IM). METHODS: We collected serial aspartate aminotransferase (AST) and alanine transaminase (ALT) levels and ultrasound-measured spleen sizes in university athletes who had been diagnosed with IM. Our study included seven university-aged athletes from a single institution. Patients received serial liver function tests (LFT) and splenic ultrasound testing until resolution of symptoms and full return to sport. The effects of AST, ALT, and days from symptom onset were analyzed using multivariable mixed-effects linear regression models. RESULTS: Levels of AST and ALT were significantly correlated with spleen size. For each 10-unit increase in AST and ALT values, spleen size increased by 0.1 cm (p = 0.007) and 0.09 cm (p = 0.008), respectively. Decreasing levels of ALT and AST correlated with a decrease in spleen size. Normalization of AST/ALT values correlated with return of spleen size to baseline. CONCLUSIONS: Liver function testing may be useful in the return-to-play decision-making process for athletes with IM.

SOHO State of the Art Updates and Next Questions | Choosing and Properly Using a JAK Inhibitor in Myelofibrosis.

Myelofibrosis (MF) is a chronic myeloid neoplasm characterized by myeloproliferation, bone marrow fibrosis, splenomegaly, and constitutional symptoms related to pro-inflammatory cytokine signaling. Biologically, MF is characterized by constitutive activation of JAK-STAT signaling; accordingly, JAK inhibitors have been rationally developed to treat MF. Following the initial approval of ruxolitinib in 2011, three additional agents have been approved: fedratinib, pacritinib, and momelotinib. As these therapies are noncurative, allogeneic stem cell transplantation remains a key treatment modality and patients with MF who are deemed candidates should be referred to a transplant center. This potentially curative but toxic approach is typically reserved for patients with higher-risk disease, and JAK inhibitors are recommended in the pretransplant setting. JAK inhibitors have proven effective at managing splenomegaly and constitutional symptoms and should be started early in the disease course in patients presenting with these clinical manifestations; asymptomatic patients may initially be followed with close surveillance. Drug-related myelosuppression has been a challenge with initial JAK inhibitors, particularly in patients presenting with a cytopenic phenotype. However, newer agents, namely pacritinib and momelotinib, have proven more effective in this setting and are approved for patients with significant thrombocytopenia and anemia, respectively. Resistance or disease progression is clinically challenging and may be defined by several possible events, such as increasing splenomegaly or progression to accelerated or blast phase disease. However, with multiple JAK inhibitors now approved, sequencing of these agents appears poised to improve outcomes. Additionally, novel JAK inhibitors and JAK inhibitor-based combinations are in clinical development.

Diagnostic insights into splenic pathologies: the role of multiparametric ultrasound.

Ultrasound (US) evaluation of the spleen is mandatory in the assessment of patients with chronic liver disease, and splenomegaly can be a sign of systemic diseases. However, due to the lack of distinctive ultrasound findings in specific splenic pathologies, clinical diagnosis can be very challenging. Splenomegaly, defined by increased splenic dimensions, can indicate underlying systemic conditions and is a common manifestation of portal hypertension (PH). Ultrasound and Doppler techniques help assessing splenic involvement in PH. Splenic stiffness measurement, using elastography, offers additional diagnostic accuracy, especially when liver stiffness measurements are inconclusive. CEUS enhances the diagnostic capability for focal splenic lesions, differentiating between benign and malignant lesions by their distinct enhancement patterns, and plays also a critical role in the context of splenic traumatic pathology. Overall, CEUS significantly improves the characterization of splenic pathology, reducing the need for invasive procedures and ensuring appropriate patient management. This review article describes the normal US findings of the spleen and examines the role of multiparametric US in the evaluation of the most common splenic pathologies encountered in the daily clinical practice.

Visceral Leishmaniasis Following A+AVD Treatment in a Patient with Classical Hodgkin's Lymphoma: A Case Report and Review of the Literature.

We present the case of a 43-year-old Caucasian man who developed visceral leishmaniasis (VL) following treatment with a combination of brentuximab vedotin and doxorubicin, vinblastine, and dacarbazine (A+AVD) for advanced-stage classical Hodgkin's lymphoma (cHL). The patient initially showed a favorable response to the treatment, but shortly after completing six cycles, he experienced recurrent fever, splenomegaly, and severe anemia. Extensive infectious disease evaluations led to a diagnosis of VL, confirmed by PCR testing. The patient was treated with amphotericin B, resulting in full clinical recovery. In addition to presenting this rare case, we conducted a full review of the literature on VL in the context of hematological disorders, including non-Hodgkin's lymphoma, splenic marginal zone lymphoma, and other lymphoproliferative diseases. This review highlights the increasing prevalence of VL in immunocompromised individuals, particularly those undergoing treatments like chemotherapy or immunotherapy, and underscores the importance of considering VL in differential diagnoses when such patients present with persistent fever and splenomegaly.

Unusual renal displacement due to massive splenomegaly: a rare case report and review of literature.

Here, the case of a female patient in her late 60s, who presented to hospital for a scheduled health check relating to a history of myelofibrosis for the previous 9 years, is described. She recently experienced weight loss and abdominal distention. Physical examination revealed no abnormality or tenderness. Laboratory examination showed decreased blood cells, platelets and haemoglobin, and normal renal function. Ultrasound and computed tomography scans revealed a massively enlarged spleen and displaced and compressed left kidney with abnormal features, but normal right kidney. The patient declined surgery and her myelofibrosis was treated with ruxolitinib, with a recommendation of annual follow-up observation. Despite many recorded cases of left renal displacement caused by splenomegaly, it is very rare for the left kidney to be pushed across the midline to the right side by an enlarged spleen. This article explores the causes and management of this uncommon condition and provides a review of previous literature reports with the aim of enhancing the understanding of unusual renal displacement due to massive splenomegaly, and its potential treatment options.

Familial hyperphosphatemic tumoral calcinosis: A rare case report from Syria.

Key Clinical Message: Tumoral calcinosis is a very rare disease mainly caused by a disturbance in phosphate metabolism. It is advisable to contemplate screening more organs such as testes, thyroid, and spleen in patients with TC. This study provides insight into tumoral calcinosis for physicians in the region and encourages future work on the matter. Abstract: Familial hyperphosphatemic tumoral calcinosis (FHTC) characterized by progressive deposition of calcium phosphate crystals in soft tissues. Tumoral calcinosis (TC) is often underdiagnosed in Syria as it cannot be confirmed without genetic testing, which is unavailable in Syria. We present the first reported case from Syria of a man with TC. This case has findings that were not reported in other cases such as testicular calcification, brain calcification, enlarged thyroid, and splenomegaly. Determining these genes in the case presented wasn't possible and future studies need to overcome this hurdle.

Change of skeletal muscle mass in cirrhotic patients with hypersplenism after partial splenic artery embolization.

PURPOSE: Partial splenic artery embolization (PSAE) is an effective procedure for cirrhotic patients with hypersplenism. The aim of our study is to evaluate the effect of PSAE on skeletal muscle, and to identify the predictor for an improvement in skeletal muscle index (SMI) in cirrhotic patients with hypersplenism after PSAE. MATERIALS AND METHODS: 466 cirrhotic patients with hypersplenism underwent PASE between Dec 2013 and Mar 2022. Medical records and CT images of enrolled patients were analyzed. RESULTS: 105 cirrhotic patients with hypersplenism were enrolled. Sarcopenia was observed in 60.00 % (63/105) of these patients, 68.25 % (43/63) of male patients, and 31.75 % (20/63) of female patients. In cirrhotic patients, no significant change in the mean SMI at the third lumbar vertebra (L3) level after PSAE. In patients with sarcopenia, the L3 SMI increased from 36.77 cm2/m2 (baseline) to 43.38 cm2/m2 (P < 0.01), the L3 subcutaneous fat area (SFA) increased from 79.16 cm2 (baseline) to 103.52 cm2 (P < 0.01) at 12-month follow-up after PSAE. In patients without sarcopenia, the L3 SMI decreased from 58.38 cm2/m2 (baseline) to 49.44 cm2/m2 (P < 0.05), the L3 SFA increased from 89.63 cm2 (baseline) to 94.77 cm2 (P > 0.05) at 12-month follow-up after PSAE. Univariate and multivariate analysis demonstrated splenic infarction rate (OR: 0.01, P = 0.0032) and SMI (OR: 0.84, P < 0.001) were independent predictors for an improvement in skeletal muscle in patients with sarcopenia. CONCLUSIONS: In cirrhotic patients with sarcopenia, an improvement in skeletal muscle and fat mass was observed after PSAE; splenic infarction rate and the L3 SMI before PSAE predicted an improvement in skeletal muscle index in patients with sarcopenia after PSAE.

Indices and ferritin level that predict organ involvement in adult-onset Still's disease.

Aim: The aim of the study is to evaluate whether C-reactive protein to albumin ratio (CAR), lactate dehydrogenase to albumin ratio (LAR), ferritin to erythrocyte sedimentation rate ratio (FER), systemic immune-inflammation index (SII), prognostic nutritional index (PNI) indices and ferritin level can predict organ involvement in adult-onset Still's disease (AOSD) patients.Methods: This study was planned as a cross-sectional study. Univariate and multivariate logistic regression analyses were performed to evaluate the usefulness of ferritin level and inflammatory indices in defining organ involvement.Results: Sixty-one patients diagnosed with AOSD were included in this study. Multivariate logistic regression analyzes showed that LAR (OR 1.028, 95% CI: 1.011-1.044) (p = 0.001) index predicted lymphadenopathy involvement, CAR (OR 1.249, 95% CI: 1.087-1.435) (p = 0.002) index predicted hepatomegaly involvement, ferritin level (OR 1.004, 95% CI: 1.001-1.008) (p = 0.007) predicted splenomegaly involvement, FER (OR 1.085, 95% CI: 1.012-1.164) (p = 0.021) and PNI (OR 0.271, 95% CI: 1.132-0.553) (p < 0.001) index predicted the occurrence of serositis.Conclusion: This study showed that ferritin level, CAR, FER, PNI and LAR markers may predict organ involvement at diagnosis in AOSD patients.

[Box: see text].

An Atypical Presentation of Sarcoidosis.

Sarcoidosis is a multisystem disease characterized by non-caseating granulomatous organ infiltration. We describe an atypical presentation of sarcoidosis in a 43-year-old male presenting with fatigue and shortness of breath. He had a preceding history of recurrent venous thromboembolism (VTE), hemolytic anemia, cirrhosis, peripheral neuropathies, and calcium deposition, which pre-dated hypercalcemia; he was later diagnosed with IgA nephropathy. Clinicians should consider sarcoidosis in patients with findings of multisystem disease even without hilar lymphadenopathy or hypercalcemia.

A Case Report of Hemophagocytic Lymphohistiocytosis Masquerading as Sepsis.

Profound inflammation due to cytokine storm is often the underlying cause of death in patients with hemophagocytic lymphohistiocytosis (HLH). Sepsis, while a precipitant, is also the great masquerader that may hide early signs of HLH. Prompt recognition is important to prevent rapid clinical decline and death. A patient presented with two weeks of unremitting fever of 103°F, dysuria, bilateral flank pain, and confusion. Obstructive uropathy and pyelonephritis were treated with a Foley catheter and antibiotics. There were abnormal developments during his hospitalization including a deep vein thrombus despite prophylactic anticoagulation. Antibiotics and Foley management did not improve fevers or renal injury so he eventually required continuous renal replacement therapy and blood product transfusions. In rapid progression, the patient developed pancytopenia, neutropenia, hyperferritinemia, hypertriglyceridemia, and hypofibrinogenemia suspicious for HLH. A bone marrow biopsy was consistent with progressive T-cell lymphoma, the likely cause of secondary HLH. Antineoplastics, corticosteroids, and opportunistic prophylaxis were pursued. Unfortunately, the cytopenias worsened, and the patient developed shock with hypoxemia and hypotension, followed by cardiac arrest and demise.

Lysinuric Protein Intolerance: Not Only a Disorder for Pediatric Nephrologists - Case Report.

INTRODUCTION: Lysinuric protein intolerance (LPI) is a multisystemic inborn error of metabolism with a variable clinical expressivity that usually begins in childhood with growth failure and gastroenterological/neurological problems related to the altered urea cycle and, later, with complications involving the renal, pulmonary, and immunohematological systems. CASE REPORT: We present the case of a 40-year-old woman suffering from chronic kidney disease in the context of a LPI, whose diagnosis was challenging because the signs of the disease were always blurred and the patient never manifested critical episodes typical of this multisystemic disease. In addition to renal disease, splenomegaly, thrombocytopenia, elevated lactate dehydrogenase (LDH), hyperferritinemia, and hypertriglyceridemia were also present. A thorough investigation of the patient's food preferences revealed her spontaneous aversion to protein-containing foods and excessive drowsiness during the occurrence of infectious episodes or on the rare occasions of excessive protein intake, although without ever coming to medical attention. These nuanced signs led us to suspect an impairment of the urea cycle and ultimately allowed us to narrow down the diagnosis to LPI through biochemical and genetic investigations. CONCLUSION: Nephrologists should consider LPI in the differential diagnosis, whenever a patient presents with mixed proteinuria, tubular dysfunction, and/or chronic kidney disease of unknown origin. In these circumstances, we suggest looking for other signs such as growth failure, signs and symptoms ascribed to urea-cycle impairment, pulmonary involvement, hepatosplenomegaly, and laboratory alterations such as pancytopenia, hyperferritinemia, lipid abnormalities, and elevated LDH.

Pseudoquiste esplénico espontáneo: Enfoque diagnóstico y terapéutico. Reporte de un caso / Spontaneous splenic pseudocyst: Diagnostic and therapeutic approach. A case report

Introducción. Los quistes esplénicos son entidades anatómicas y clínicas poco frecuentes, con una incidencia aproximada de 0,07 %. Se clasifican como quistes esplénicos primarios, que contienen revestimiento epitelial y se subdividen en parasitarios y no parasitarios según su etiología, y quistes secundarios, que no poseen revestimiento epitelial en la luz quística y suelen ser el resultado de un traumatismo abdominal. Por lo general, son asintomáticos y se pueden encontrar de manera incidental durante estudios de imagen o en cirugía. Los síntomas están relacionados con el tamaño de los quistes. El tratamiento ideal es la resección quirúrgica, que puede ser total o parcial. Caso clínico. Paciente femenina de 23 años, sin antecedentes de trauma, con dolor abdominal intermitente, de varios meses de evolución. En los estudios imagenológicos se identificó un pseudoquiste esplénico gigante. Fue tratada mediante esplenectomía total por laparoscopia, sin complicaciones quirúrgicas. Resultados. Tuvo una adecuada evolución postoperatoria. Conclusión.El diagnóstico de quiste esplénico se realiza mediante estudios imagenológicos y se confirma con el análisis histopatológico. La esplenectomía total ha sido el tratamiento tradicional; sin embargo, ahora mediante la implementación de abordajes mínimamente invasivos, se prefiere la esplenectomía parcial, con el fin de preservar tejido esplénico y su función inmunológica.

Introduction.Splenic cysts are rare anatomical and clinical entities, with an approximate incidence of 0.07%. They are classified as primary splenic cysts, which contain epithelial lining and are subdivided into parasitic and non-parasitic depending on their etiology, and the secondary splenic cysts, which do not have an epithelial lining in the cystic lumen and are usually the result of abdominal trauma. They are usually asymptomatic and can be found incidentally during imaging studies or in surgery. The symptoms are related to the size of the cysts. The ideal treatment is surgical resection, which can be total or partial. Clinical case. A 23-year-old female patient, with no history of trauma, with intermittent abdominal pain, lasting several months. Imaging studies identified a giant splenic pseudocyst. She was treated by total laparoscopic splenectomy, without surgical complications. Results. She had an adequate postoperative evolution. Conclusion. The diagnosis of splenic cyst is made through imaging studies and confirmed with histopathological analysis. Total splenectomy has been the traditional treatment; however, now through the implementation of minimally invasive approaches, partial splenectomy is preferred, in order to preserve splenic tissue and its immunological function.

Pediatric Gaucher Disease Type 3 Presenting with Oculomotor Apraxia: A Case Report.

We report on a 4-year-old boy affected by Gaucher disease (GD) type 3, who presented with splenomegaly and a history of oculomotor apraxia. GD is a rare lysosomal storage disorder caused by glucocerebrosidase deficiency with multi-organ involvement. Besides common clinical features such as hepatosplenomegaly and skeletal involvement, less frequent neurological symptoms, such as oculomotor apraxia, are indicative of neuronopathic forms of the disease, namely GD type 3, to be confirmed both by enzyme activity and genetic testing. Overall, GD management requires a multidisciplinary approach involving metabolic pediatricians, neurologists, psychologists, and geneticists, and currently relies on early enzyme replacement therapy. Although enzyme replacement therapy has proved to be effective in improving systemic signs and symptoms, it is unable to alleviate neurological complications once these have occurred, as it does not pass across the blood-brain barrier. Neurological improvements may occur through indirect mechanisms. Thus, our case report aims to highlight the importance of considering GD in the differential diagnosis of pediatric patients presenting with splenomegaly associated with neurological manifestations, as early intervention may significantly modify the disease progression and prevent further irreversible complications.

Factors Affecting an Increase in Spleen Volume and Association of Spleen Volume Variation with the Clinical Outcomes of Atezolizumab and Bevacizumab Treatment for Hepatocellular Carcinoma: A Retrospective Analysis.

INTRODUCTION: Gastrointestinal varices rupture is considered to be prone to occur during atezolizumab and bevacizumab (Atez/Bev) treatment. This study aimed to investigate predictive factors affecting the increase in spleen volume (SpV) and the association of SpV variation with the clinical outcomes of Atez/Bev. METHODS: A total of 164 HCC patients were included in this retrospective multicenter study. We measured SpV based on CT scans obtained before treatment and at evaluations. We used the inverse probability of treatment weight to address the imbalance between patient characteristics. RESULTS: The median pretreatment SpV was 184 (130-257) cm3 and the median SpV variation was 27 (9-60) cm3. An increase in the SpV was observed in 140 patients (85.4%). Age <74 years (p = 0.03), mALBI grade 2b or 3 (p = 0.03), and pretreatment SpV ≥184 cm3 (p < 0.001) were significantly associated with increased SpV. There were no significant differences in progression-free survival (PFS) or overall survival (OS) between patients with SpV variation <25 cm3 and those with SpV variation ≥25 cm3 in the crude (p = 0.3 and 0.7) and IPTW-weighted cohorts (p = 0.08 and 0.8, respectively). Regarding pretreatment SpV, there were no significant differences in PFS or OS between patients with and without pretreatment spleen enlargement in the crude (both p = 0.3) and IPTW-weighted cohort (p = 0.6 and 0.3, respectively). CONCLUSION: Caution is warranted to detect the aggravation of portal hypertension when administering Atez/Bev to young patients or patients with an impaired liver function or pretreatment spleen enlargement. The impact of spleen modulation by Atez/Bev appears to be limited on clinical efficacy.

Myelofibrosis and anemia: A German claims data analysis to describe epidemiology and current treatment.

OBJECTIVES: There is limited data on the incidence, prevalence, and treatments for myelofibrosis (MF) in Germany. This retrospective study examined claims data from 3.3 million insured individuals, spanning from 2010 to 2021. METHODS: Four sensitivity scenarios were explored to identify cases of MF. Point prevalence and cumulative incidence of MF were determined as of December 31, 2021, and within 2021, respectively. A cross-sectional analysis used the main scenario definition of MF to identify cases and evaluate the period prevalence of patients receiving treatment for symptoms and/or splenomegaly, including first-line (1L) Janus kinase inhibitor (JAKi), second-line, or further (2L+) MF-related treatment therapies during 2021. The prevalence of anemia treatment was also reported. RESULTS: The estimated standardized point prevalence of MF on December 31, 2021, was 9.9-12.4 cases per 100 000 persons, and cumulative incidence in 2021 was 1.2-1.8 cases per 100 000 persons. Standardized period prevalence in 2021 for MF patients receiving 1L JAKi and/or 2L+ MF-related treatment was 4.0 cases per 100 000. Among these patients, 47.1%-53.7% required treatment for anemia, resulting in a period prevalence of 1.9-2.2 cases per 100 000 individuals. CONCLUSION: The data reveal gaps in MF treatments and the need to improve patient quality of life.

Sarcoidosis presenting as isolated massive splenomegaly: A case report.

Key Clinical Message: Sarcoidosis is a systemic granulomatous disease with an unknown cause, marked by the presence of noncaseating granulomas in the affected organs. While the pulmonary interstitium is most frequently involved, the disease can affect almost any other organ system. Extrapulmonary involvement can occur with or without lung involvement, but isolated extrapulmonary involvement is a rare event. Isolated splenomegaly is very rare and presents an uncommon manifestation of sarcoidosis, its diagnoses is a challenge due to a broad differential diagnosis. Here, we present an intriguing case of a 28-year-old male with isolated splenic sarcoidosis. Abstract: Sarcoidosis is a systemic disease of unknown cause, marked by the presence of noncaseating granulomas in affected organs. It most frequently impacts the pulmonary interstitium, though it can also affect nearly any other organ system. This involvement can occur with or without lung involvement, but isolated extrapulmonary cases are observed in only about 10% of instances. Furthermore, isolated splenomegaly is an exceptionally rare event and an uncommon presentation of sarcoidosis, posing a significant clinical challenge due to the wide differential diagnosis. Potential differential diagnoses include hematologic cancers, primary or metastatic splenic tumors, infiltrative diseases, inflammatory conditions, and infections. We present a noteworthy case of a 28-year-old with isolated splenic sarcoidosis.

Clinical Features of Cytokine Storm Syndrome.

Cytokine storm syndrome (CSS) is a severe life-threatening condition characterized by a clinical phenotype of overwhelming systemic inflammation, hyperferritinemia, hemodynamic instability, and multiple organ failure (MOF), and, if untreated, it can potentially lead to death. The hallmark of CSS is an uncontrolled and dysfunctional immune response involving the continual activation and expansion of lymphocytes and macrophages, which secrete large amounts of cytokines, causing a cytokine storm. Many clinical features of CSS can be explained by the effects of pro-inflammatory cytokines, such as interferon (IFN)-γ, tumor necrosis factor (TNF), interleukin (IL)-1, IL-6, and IL-18 [1-7]. These cytokines are elevated in most patients with CSS as well as in animal models of CSS [8, 9]. A constellation of symptoms, signs, and laboratory abnormalities occurs that depends on the severity of the syndrome, the underlying predisposing conditions, and the triggering agent.