T-ALL presenting with i-TLP-like indolent clinical course with repeated spontaneous regressions.

Rapidly progressing ALL, a potentially fatal disease, demands timely diagnosis and treatment. On the other hand, spontaneous remission/regression (SR) is reported in various cancers including aggressive tumors like ALL. Infection or trauma-mediated immune system activation is assumed to cause SR, with the duration in cases of ALL typically being short. Indolent T-lymphoblastic proliferation (i-TLP) exhibits the uniform proliferation of TdT-positive T-cells, despite being a non-neoplastic disease, underscoring the significance of distinguishing it from T-cell acute lymphoblastic leukemia (T-ALL). i-TLP is expected to gain wider recognition and further advancements in understanding its pathology. Here, we present the case of a 59-year-old woman with T-ALL characterized by cycles of progression and SR followed by a rapid blast proliferation. This is the first reported case of T-ALL with repeated SR for more than one year, making this case an extremely rare clinical presentation. This challenging case will enhance comprehension of T-cell tumor pathogenesis.

Haematopoietic regeneration by HLA-A*0206-deficient clones in severe aplastic anaemia without definitive immunosuppressive treatment.

We describe the case of a 74-year-old man with severe aplastic anaemia who experienced persistent remission attributed to proliferation of HLA allele-deficient clones. Despite an initial worsening of pancytopenia with eltrombopag and ciclosporin treatment, gradual trilineage haematopoietic recovery occurred, with blood counts normalizing over 3 years. Flow cytometry and deep nucleotide sequencing revealed that haematopoiesis was primarily supported by several clones with somatic mutations that inactivated antigen presentation via HLA-A*0206. This suggests that monitoring haematopoietic regeneration by immune escape clones could be an alternative approach for immune aplastic anaemia patients who possess HLA allele-deficient clones and cannot tolerate standard therapy.

Case report: Spontaneous improvement and treatment considerations in leukoencephalopathy with calcifications and cysts.

We present the case of a patient with leukoencephalopathy with calcifications and cysts (LCC), who experienced progressive severe hemiparesis despite multiple neurosurgical interventions of a large contralateral cyst. Bevacizumab was proposed as an ultimate treatment option based on prior case reports. While awaiting reimbursement approval for bevacizumab, major improvement occurred in both clinical and radiological disease manifestations. The disease course of LCC is variable and unpredictable; neurosurgical treatment should be reserved for severe and progressive neurological deficits. Bevacizumab has been reported as a promising alternative treatment option. Importantly, in our case the observed clinical improvement would have been attributed to the effects of bevacizumab, if started when requested. Our case underscores the need for a natural history study for LCC and the necessity of validating treatment efficacy by systematic evaluation through appropriate clinical trials rather than relying on anecdotal evidence from published case reports.

Spontaneous Cushing's Disease Remission Induced by Pituitary Apoplexy.

Spontaneous remission of Cushing's disease (CD) is uncommon and often attributed to pituitary tumor apoplexy. We present a case involving a 14-year-old female who exhibited clinical features of Cushing's syndrome. Initial diagnostic tests indicated CD: elevated 24h urinary cortisol (235 µg/24h, n < 90 µg/24h), abnormal 1 mg dexamethasone overnight test (cortisol after 1 mg dex 3.4 µg/dL, n < 1.8 µg/dL), and elevated adrenocorticotropic hormone concentrations (83.5 pg/mL, n 10-60 pg/mL). A pituitary adenoma was suspected, so a nuclear MRI was performed, with findings suggestive of a pituitary microadenoma. The patient was referred for a transsphenoidal resection of the microadenoma. While waiting for surgery, the patient presented to the emergency department with a headache and clinical signs of meningism. A computed axial tomography of the central nervous system was performed, and no structural alterations were found. The symptoms subsided with analgesia. One month later, she presented again to the emergency department with clinical findings of acute adrenal insufficiency (cortisol level of 4.06 µg/dL), and she was noted to have spontaneous biochemical remission associated with the resolution of her symptoms of hypercortisolism. For that reason, spontaneous CD remission induced by pituitary apoplexy (PA) was diagnosed. The patient has been managed conservatively since the diagnosis and remains in clinical and biochemical remission until the present time, after 10 months of follow-up. There are three unique aspects of our case: the early age of onset of symptoms, the spontaneous remission of CD due to PA, which has been rarely reported in the medical literature, and the fact that the patient presented a microadenoma because there are fewer than 10 clinical case reports of PA associated with microadenoma.

Spontaneous Remission of Acute Lymphoblastic Leukemia Following Candida tropicalis Fungemia.

Spontaneous remission (SR) in acute lymphoblastic leukemia (ALL) is a poorly understood phenomenon that has been sporadically reported in medical literature for over a century, and the molecular and immunologic mechanisms of remission pose interesting clinical questions. Furthermore, the often-transient nature of these remissions poses a challenge to physicians in formulating an approach to treatment. We report on a rare case of Candida tropicalis sepsis in a three-year-old female with high-risk ALL who received less than two months of treatment prior to sepsis and subsequent SR.

Trend in predictive factors of choledocholithiasis: the key to the management of pediatric patients with suspected gallstones. / Tendencia en los factores predictivos de coledocolitiasis: la clave para el manejo de pacientes pediátricos con sospecha de cálculos en la vía biliar.

OBJECTIVES: To reduce the overuse of magnetic resonance cholangiopancreatography and the rates of non-therapeutic endoscopic retrograde cholangiopancreatography in pediatric patients suspected of choledocholithiasis. MATERIALS AND METHODS: Retrospective study of patients suspected of choledocholithiasis between January 2010 and June 2023. Patients with cholangitis or two or more of the following predictive factors of choledocholithiasis in initial laboratory tests and ultrasound were categorized as high-risk group: total bilirubin level ≥ 2 mg/dl, common bile duct > 6 millimeters on ultrasound; and detection of choledocholithiasis by ultrasound. Patients were recategorized according to the results of the second set of laboratory and ultrasound analysis. Confirmatory modalities (magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography, and/or intraoperative cholangiography) were used to evaluate the presence of choledocholithiasis. Finally, we assessed the predictive capability of both the initial high-risk group and the group after recategorization. RESULTS: A total of 129 patients were included. After initial studies, 72 (55.8%) patients were classified into the high-risk group. After recategorization, only 29 (22.5%) patients were included in this group. The sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy of the initial high-risk group were 89.3%, 53.5%, 34.7%, 94.7%, and 61.2%, respectively, while after recategorization, they were 82.1%, 94.1%, 79.3%, 95.0%, and 91.5%, respectively. CONCLUSIONS: Recategorization of the risk of choledocholithiasis would significantly improve the diagnostic accuracy of choledocholithiasis and help reduce the overuse of more complex and unnecessary studies/procedures.

OBJETIVOS: Disminuir la sobre indicación de la colangiorresonancia y las tasas de colangiopancreatografía retrógrada endoscópica o terapéuticas en pacientes pediátricos con sospecha de coledocolitiasis. MATERIAL Y METODOS: Estudio retrospectivo de pacientes con sospecha de coledocolitiasis entre enero de 2010 y junio de 2023. Los pacientes con colangitis o dos o más de los siguientes factores predictivos de coledocolitiasis en las pruebas de laboratorio y ecografía iniciales, se categorizaron como grupo de alto riesgo: nivel de bilirrubina total ≥ 2 mg/dl, colédoco > 6 milímetros en ecografía; y la detección de coledocolitiasis por ecografía. Los pacientes fueron recategorizados de acuerdo a los resultados del segundo conjunto de análisis de laboratorio y ecografía. Para evaluar la presencia de coledocolitiasis se utilizaron modalidades confirmatorias (colangiorresonancia, colangiopancreatografía retrógrada endoscópica y/o colangiografía intraoperatoria). Finalmente, evaluamos la capacidad predictiva tanto del grupo de alto riesgo inicial como del grupo después de la recategorización. RESULTADOS: Se incluyeron 129 pacientes. Luego de los estudios iniciales, 72 (55,8%) pacientes se clasificaron en el grupo de alto riesgo. Luego de la recategorización, solo 29 (22,5%) pacientes fueron incluidos dentro de este grupo. La sensibilidad, especificidad, valor predictivo positivo, valor predictivo negativo y precisión diagnóstica del grupo de alto riesgo inicial fueron de 89,3%, 53,5%, 34,7%, 94,7% y 61,2%, mientras que luego de la recategorización fueron de 82,1%, 94,1%, 79,3%, 95,0% y 91,5%, respectivamente. CONCLUSIONES: La recategorización del riesgo de coledocolitiasis, mejoraría significativamente la precisión diagnóstica de coledocolitiasis y ayudaría a disminuir la sobre indicación de estudios/procedimientos complejos e innecesarios.

Baló's concentric sclerosis with spontaneous remission and favorable prognosis.

Introduction: Baló's concentric sclerosis (BCS) is a rare type of central nervous system demyelinating disorder. Most patients with BCS are treated with corticosteroids, and spontaneous remission has seldom been described. Case presentation: A 46-year-old man presented with a subacute-onset headache and memory loss. Brain magnetic resonance imaging (MRI) revealed multiple onion-shaped ring lesions with mild enhancement in the outermost ring. A brain biopsy revealed significant myelin loss. The diagnosis of BCS was established based on the MRI results and pathological findings. Interestingly, the patient recovered almost completely without immunotherapy, with repeated brain MRI at the 1-year follow-up showing an obvious reduction in the extent of the lesions. Conclusion: Neurologists should improve the recognition of the typical MRI features of BCS to avoid unnecessary biopsies. Although rare, spontaneous remission can be observed in clinical practice.

Spontaneous remission of a pulmonary sclerosing epithelioid fibrosarcoma: a case report of a possible abscopal effect.

Background: Spontaneous remission (SR) is defined as the complete or partial disappearance of a diagnosed malignant disease in the absence of known active medical treatment. The role of the immune system is thought to be important, but has not yet been elucidated. On this matter, there are studies that suggest that the abscopal effect (AE), which is defined as the remission of untreated lesions beyond the irradiated area, may be explained by the activation of a systemic immune response against the tumor. Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of soft tissue sarcoma that is characterized by a slow evolution, with local recurrences and late metastases. The treatment is based on surgery, leaving a minimal role to chemotherapy (ChT) and radiotherapy (RT) for metastatic unresectable disease, and no cases of SR have been reported in the literature so far. Case Description: We present the case of a patient with a lung metastatic recurrence of SEF, diagnosed and treated with surgery 8 years before. After progression to pazopanib and other ChT drugs, because of the chest pain associated with a pleural mass invading the second costal arch, the patient received antalgic local RT treatment. Months later, and without any further treatment, a partial remission of all the tumoral lesions was presented, and she is alive 25 years after the first diagnosis. Conclusions: As far as reported in the literature, this is the first case of SR in SEF. Among the possible causes of this SR, we think that the most plausible is that palliative treatment with RT of the pleural mass induced an AE, leading to a reduction of all tumoral lesions, even those outside the irradiated region.

Spontaneous Remission of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report.

Spontaneous remissions (SRs) in blastic plasmacytoid dendritic cell neoplasms (BPDCNs) are infrequent, poorly documented, and transient. We report a 40-year-old man presenting with bycitopenia and soft tissue infection. The bone marrow exhibited 3% abnormal cells. Immunophenotyping of these cells revealed the antigens CD45+ (dim), CD34+, CD117+, CD123+ (bright), HLA-DR+ (bimodal), CD56+ (bright), CD33+, CD13+, CD2+, and CD22+ (dim) and the partial expression of the CD10+, CD36+, and CD7+ antigens. All other myeloid, monocytic, and lymphoid antigens were negative. Genetic studies showed a complex karyotype and mutations in the TP53R337C and KRASG12D genes. On hospital admission, the patient showed a subcutaneous nodule on the right hand and left lower limb. Flow cytometry multiparameter (FCM) analysis showed the presence of 29% abnormal cells with the previously described immunophenotype. The patient was diagnosed with BPDCN. The patient was treated with broad-spectrum antibiotics for soft tissue infection, which delayed therapy for BPDCN. No steroids or chemotherapeutic or hypomethylating agents were administered. His blood cell counts improved and skin lesions disappeared, until the patient relapsed five months after achieving spontaneous remission. About 60% of abnormal cells were identified. No changes in immunophenotype or the results of genetic studies were observed. The patient underwent a HyperCVAD chemotherapy regimen for six cycles. Consolidation therapy was performed via allogeneic bone marrow transplantation with an HLA-unrelated donor. One year after the bone marrow transplant, the patient died due to the progression of his underlying disease, coinciding with a respiratory infection caused by SARS-CoV-2. In the available literature, SRs are often linked to infections or other stimulators of the immune system, suggesting that powerful immune activation could play a role in controlling the leukemic clone. Nevertheless, the underlying mechanism of this phenomenon is not clearly understood. We hypothesize that the immune system would force the leukemic stem cell (LSC) to undergo a state of quiescence. This loss of replication causes the LSC progeny to die off, resulting in the SR of BPDCN.

Spontaneous Remission of Minimal Change Disease in a Colon Cancer Patient: A Case Report.

Introduction: Minimal change disease (MCD) is most often primary but may occur secondary to other systemic diseases such as malignancy. In secondary MCD, spontaneous remission of nephrotic syndrome after the treatment of related diseases without steroid therapy is rare. Case Presentation: A 78-year-old man visited the outpatient clinic with foamy urine and generalized edema that had persisted for 2 months. The patient had nephrotic syndrome. Before a kidney biopsy, he underwent several tests to determine the secondary cause of the nephrotic syndrome. The serum CEA was slightly elevated, and colon cancer was detected in the sigmoid colon. MCD was diagnosed from a kidney biopsy. He immediately underwent surgery for colon cancer. Complete remission of the MCD was achieved within 2 weeks after surgery. Conclusion: Here, we report a rare case of a patient with secondary MCD who successfully achieved spontaneous remission after colon cancer surgery.

IgG4-related Disease: Recent Topics on Immunological Aspects of This Disorder and Their Application in New Treatment Strategies.

IgG4-related disease (IgG4-RD) is a systemic and chronic inflammatory disorder that can affect every part of the body. The formation of tertiary lymphoid tissues (TLT) in the affected organs may be a key phenomenon in understanding the pathogenesis of this disease because T follicular helper (Tfh) 2 cells play an important role in IgG4 class switching within TLT in the affected organs or tissues. TLT formation leads to the formation of masses or swelling of the affected organs. Interleukin (IL)-4 and IL-10 are critical cytokines for IgG4-class switching and are produced in TLT. Other factors, such as CD4-positive (CD4+) cytotoxic T cells, M2 macrophages, and LAG3+ Tfh cells, have been identified as disease-specific contributors to lesion formation. In this review, I describe the current knowledge necessary to understand the pathogenesis of this disease and recent developments in treatment strategies beyond B-cell depletion therapy.

Case Report: Immune dysregulation associated with long-lasting regression of a (pre)leukemic clone.

Regression of leukemia in the absence of disease-modifying therapy remains poorly understood, although immunological mechanisms are thought to play a role. Here, we present a unique case of a 17-year-old boy with immune dysregulation and long-lasting regression of a (pre)leukemic clone in the absence of disease-modifying therapy. Using molecular and immunological analyses, we identified bone marrow features associated with disease control and loss thereof. In addition, our case reveals that detection of certain fusion genes with hardly any blasts in the bone marrow may be indicative of an accompanying oncogenic fusion gene, with implications for disease surveillance- and management in future patients.

Spontaneous Remission in a Patient with Chronic Myeloid Leukemia: A Case Report.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm in which granulocytic cells are the main proliferative component. At diagnosis, more than 90% of CML cases have the characteristic Philadelphia chromosome, containing the BCR::ABL1 fusion gene. The natural history of untreated CML is an initial indolent chronic phase which will be followed by an accelerated phase, blast phase, or both. Tyrosine kinase inhibitors (TKIs) have dramatically altered the natural history of CML. TKI discontinuation with the goal of treatment-free remission is currently part of current management recommendations. However, spontaneous remission without receiving any treatment is extraordinarily rare in CML patients. Herein, we report a 56-year-old male who presented with leukocytosis and was diagnosed as a case of CML in the chronic phase; however, treatment with TKIs was not initiated due to spontaneous hematological as well as molecular remission.

Spontaneous remission of skull Langerhans cell histiocytosis that had developed by repeated head injury: illustrative case.

BACKGROUND: Langerhans cell histiocytosis (LCH) was previously characterized as the proliferation of Langerhans-type histiocytes with a wide range of clinical presentations that arise mostly in children. The typical presentation is a gradually enlarging, painless skull mass. Rapid clinical deterioration is rare. OBSERVATIONS: A 3-year-old boy who had incurred a right frontal impact head injury demonstrated no apparent neurological deficits. He subsequently bruised the same region multiple times. The right frontal swelling gradually increased over the course of 6 days after the initial injury. Skull radiography showed no bony lesion. The same site enlarged markedly 12 days after the initial injury. Magnetic resonance imaging revealed a frontal bony tumorous lesion associated with multiple subcutaneous cystic mass lesions. The patient underwent open biopsy of the skull lesion and evacuation of the subcutaneous lesions. Histopathological examination confirmed the diagnosis of LCH. Immunohistochemical evaluation revealed positivity for CD1a and langerin and no immunopositivity for BRAF V600E. The skull lesion spontaneously disappeared 30 days after the biopsy without recurrence. LESSONS: Physicians should be aware of this rare clinical manifestation of LCH that developed by a repeat head injury.

[A late onset diagnosis of giant cell arteritis in remission]. / Diagnostic tardif d'une artérite à cellules géantes en rémission.

Giant-cell arteritis (GCA) is a classical cause of chronical inflammation (CI) in the elderly, causing headaches, scalp hypersensitivity and jaw claudication. We describe a patient with a GCA revealed with a year-long biological inflammation and weight loss. Diagnosis was performed on a systematic temporal artery biopsy showing typical histological features. No treatment was intended as the patient had a spontaneous remission, maintained at one year of follow-up. This case highlights the benefit of a systematic temporal artery biopsy to explore CI and reminds us that GCA may undergo spontaneous remission.

Myelin oligodendrocyte glycoprotein antibody-associated disease in a patient with symptoms of aseptic meningitis who achieved spontaneous remission: A case report and review of the literature.

BACKGROUND: A few case reports have described patients with myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated demyelinating syndrome who presented with symptoms of aseptic meningitis. All such patients required immunotherapy. We report a patient with MOG-Ab-associated disorder (MOGAD) who presented with symptoms of aseptic meningitis and improved without treatment. CASE: A 13-year-old girl presented with fever, headache, decreased appetite, and neck stiffness. Cerebrospinal fluid (CSF) analysis revealed pleocytosis and magnetic resonance imaging (MRI) showed leptomeningeal enhancement. The patient was diagnosed with aseptic meningitis at admission. However, there were no signs of recovery 4 days after admission (i.e., 8 days after disease onset). Therefore, we performed extensive investigations to identify the cause of the underlying infection and inflammation. On day 14 after admission, the serum MOG-Ab test performed at admission came back positive (1:128) and she was diagnosed with MOGAD. She was discharged on day 18 after admission, because her symptoms, CSF pleocytosis, and MRI findings had improved. About 6 weeks after discharge, MRI revealed hyperintensity without gadolinium enhancement. However, her serum MOG-Ab test was negative. We did follow-ups for 11 months but found no new neurological symptoms. DISCUSSION AND CONCLUSION: To the best of our knowledge, this is the first ever report of a pediatric patient with MOGAD experiencing spontaneous remission with no demyelinating symptoms during an extended follow-up period.

A 15-Year-Old Boy with Primary Maxillary Bone Anaplastic Lymphoma Kinase-Positive Anaplastic Large Cell Lymphoma Relapsed with Rib Metastasis after Spontaneous Remission of a Maxillary Bone Lesion: A Case Report and Literature Review.

Anaplastic large cell lymphoma (ALCL) is a rare form of non-Hodgkin's lymphoma (NHL) in children, accounting for 10-15% of all NHL cases. ALCL is currently classified as follows: systemic anaplastic lymphoma kinase (ALK)-positive, systemic ALK-negative, primary cutaneous, and breast implant-associated ALCL. In children, systemic ALK-positive ALCL is the most common, and patients often present with extranodal involvement. We report a rare case of systemic ALK-positive ALCL with primary bone involvement in a 15-year-old male patient. Primary bone lymphoma is most commonly observed in diffuse large B-cell lymphoma and is extremely rare in systemic ALCL. Therefore, the clinical features and prognosis of primary bone ALCL remain unclear. Our patient had spontaneous remission of primary maxillary bone ALCL after gingival scraping but relapsed 12 months later with rib metastasis. Spontaneous remission of ALCL has been reported frequently in primary cutaneous ALCL and rarely in systemic ALCL. Our case demonstrates for the first time that systemic ALCL can also present as solitary bone involvement that can spontaneously remit. Because systemic ALCL is aggressive and has a risk of relapse, as in our case, it is important to consider ALCL in the differential diagnosis of primary bone lesions and to make a precise pathological diagnosis.

Repeated spontaneous remission of acute myeloid leukemia in response to various infections: a case report.

BACKGROUND: Acute myeloid leukemia (AML) is a progressive hematological malignancy that can be fatal when left untreated. However, spontaneous remission is rarely observed in the presence of infectious diseases. CASE PRESENTATION: We treated an 80-year-old woman with AML who spontaneously underwent remission after infections. Spontaneous remission was observed after each of three independent clinical infections caused by different pathogens-nontuberculous Mycobacterium infection, pulmonary aspergillosis, and Escherichia coli bacteremia. All infections were treated promptly with antimicrobials. Mycobacterium avium infection was treated with azithromycin, rifampin, and ethambutol. Pulmonary aspergillosis was treated with itraconazole followed by voriconazole. E. coli infection was treated with meropenem. During each infectious episode, leukemic cells disappeared from the patient's peripheral blood and pancytopenia improved without routine blood transfusion. These clinical effects lasted for several months. The patient has survived for > 2 years beyond the median survival time of end-stage AML. Thus, this case represents an immunological antileukemic effect of systemic infections. CONCLUSIONS: We have discussed a common mechanism of spontaneous remission of AML without chemotherapy, clinically exhibited by infection immunology. We believe that infections exert a limited immunological effect against AML, which may prolong survival among elderly individuals with AML.