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Abstract Background Undifferentiated arthritis (UA) is a term used to describe patients with inflammatory arthritis that has not differentiated into a specific rheumatic disease. UA may be a pre-stage of rheumatoid arthritis (RA) or another inflammatory disease or remain undifferentiated, but a substantial proportion of patients may also achieve spontaneous remission. As UA may be an early presentation of RA, rheumatologists often start methotrexate (or another csDMARD) as early as possible. There are however very little data on the potential benefits of early DMARD treatment, and longitudinal data suggests that long-term outcomes such as physical functioning hardly improved in these patients in the past decades. In the I CEA trial, we investigate if it is beneficial to start early treatment with MTX or baricitinib, a more rapidly acting drug with a broader mechanism of action, compared to waiting for spontaneous remission with symptomatic therapy in patients with UA. Methods The I CEA is a multicenter single-blind (independent assessor) randomized clinical trial with a 3-month interventional and 9-month observational follow-up period. The study includes patients with early (

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.

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.

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.

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.

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.

Abstract 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.

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.

Abstract Introduction Over half of the patients with hepatitis B virus associated membranous nephropathy (HBV-MN) were found to be phospholipase A2 receptor (PLA2R) positive. Whether MN is really secondary to hepatitis B or just coincidence of hepatitis and PLA2R positive idiopathic MN (IMN) remains controversial. Methods We retrospectively studied seven PLA2R positive HBV-MN patients with complete data in Huashan Hospital from 2009 to 2016 and compared them with PLA2R positive idiopathic MN patients. Results Proteinuria and renal function of these 7 HBV-MN patients were similar to that of IMN patients. However, 5 of them were female and half showed hypocomplementemia, while in IMN group only 32.4% were female and 20% had hypocomplementemia, and the level of hematuria was 94.5/μL in HBV-MN patients and 64.9 /μL in IMN patients, though there was no statistically significant difference. Renal biopsies revealed significantly increased mesangial eletron-deposits in HBV-MN patients. All 7 patients received antiviral therapy, and one patient received immunosuppresants due to severe nephrotic syndrome with acute myocardial infarction and elevated serum creatinine. Compared with IMN group, the prevalence of remission without immunosuppressive therapy of HBV-MN patients was higher (85.7% vs. 43.7%), while the percentage of patients receiving immunosuppresants was lower (14.3% vs. 47.9%) (P=0.048). Conclusion Compared with IMN patients, PLA2R positive HBV-MN patients had a more favorable prognosis after antiviral therapy, indicating a secondary form of MN. For these patients, antiviral treatment is recommended and long observation time should be provided before use of immunosuppressive treatment.

Spontaneous remission (SR) of local recurrence after adjuvant immunotherapy has rarely been reported, and the underlying mechanism is poorly understood. Herein, we reported a patient with stage cT2aN2M0 squamous cell lung carcinoma who received neoadjuvant and adjuvant treatment with nivolumab plus chemotherapy. The patient experienced a late relapse in the subcarinal lymph node seven months after the last dosage of treatment but achieved SR in the next three months without additional antitumor therapy. The complete response lasted for eleven months and counting. Notably, high copies of pathogenic microorganisms were detected in the patient’s bronchoalveolar lavage fluid along with the recurrence but disappeared after SR. The patient also experienced a lymph node puncture-induced fever but had no other symptoms. A longitudinal analysis of infiltrated immune cells in the recurrent lymph node was performed by multiplex immunofluorescence and whole transcriptome sequencing, which revealed that CD8+ T cells were recruited during the initial relapse, specifically in the stromal area, then migrated into the tumor tissue, and continued to increase after elimination of tumor cells. Meanwhile, the initial recruitment of CD8+ T cells was coupled with a higher proportion of B cells, and the abundant neutrophil population was synchronous with the infiltration of CD8+ T cells into tumor cells. This is the first report on an Non-small cell lung cancer (NSCLC) patient with a late relapse after adjuvant immune checkpoint inhibitor (ICI) therapy who achieved SR. Our case highlights the complexity and plasticity of antitumor immunity and is expected to help find efficient strategies against the resistance of ICI treatment.

Objective(s): Only few studies have assessed the use of gallium citrate-67 single-photon emission computed tomography/computed tomography (67Ga-SPECT/CT) for localizing the foci of classic fever of unknown origin (FUO) and inflammation of unknown origin (IUO). Hence, the current study aimed to assess the diagnostic contribution of 67Ga-SPECT/CT in a tertiary referral setting where nuclear imaging tests are performed after an unsuccessful comprehensive primary diagnostic workup.Methods: We retrospectively assessed the medical records of 27 adult patients with FUO/IUO who had an unsuccessful diagnostic workup and who underwent 67Ga-SPECT/CT for the localization of FUO/IUO foci in our university hospital between 2013 and 2019. The primary outcome was diagnostic yield. The secondary outcomes were overall clinical efficacy and spontaneous remission of FUO/IUO symptoms in patients with a negative 67Ga-SPECT/CT finding.Results: Almost all patients completed the recommended diagnostic workup, except for urine culture and abdominal ultrasonography. Moreover, prior to 67Ga-SPECT/CT, all patients underwent thoraco-abdominopelvic CT scan, which was a non-diagnostic procedure. After a median follow-up of 843 days, the cause was identified in 16 (59%) patients. 67Ga-SPECT/CT successfully localized the FUO/IUO foci in eight patients (diagnostic yield = 30%; 95% confidence interval [CI]: 14%–50%). However, the causes remained unknown during follow-up in 11 (41%) patients. Among them, five experienced spontaneous regression of symptoms. 67Ga-SPECT/CT was negative in four of the five patients with spontaneous regression in symptoms without a definite cause. Considering this an important event, the overall clinical efficacy of 67Ga-SPECT/CT increased to 44% (95% CI: 25%–65%).Conclusion: 67Ga-SPECT/CT had an acceptable diagnostic yield for the localization of FUO/IUO foci, which are challenging to diagnose, in a contemporary tertiary referral care setting. In patients who experienced spontaneous regression in symptoms with an unexplained cause, the absence of abnormal uptake might indicate prospective spontaneous remission. Thus, 67Ga-SPECT/CT could be an active first-line nuclear imaging modality in settings where fluorine-18-fluorodeoxy glucose positron emission tomography and computed tomography is not available for the assessment of FUO/IUO causes.

Abstract Background Behçet’s disease (BD) is a systemic inflammatory vasculitis with both autoimmune and autoinflammatory properties. Renal involvement in BD and its spontaneous remission have been rare. We herein describe a case of parallel disease activity of BD with entero and renal involvements, followed by a spontaneous remission without corticosteroid treatment. Case presentation A 54-year-old woman who had a 4-year history of BD, maintained with colchicine treatment, suffered abdominal pain, hemorrhagic stool and diarrhea. Physical examination revealed strong tenderness in the entire abdomen. Laboratory test results showed increased levels of inflammation, and a computed tomography scan revealed edematous intestinal wall thickening with ascites. Blood and stool cultures showed no specific findings. Since she was suspected to have developed panperitonitis with acute enterocolitis, she started treatment with an antibacterial agent under bowel rest. Her abdominal symptoms gradually improved, while diarrhea and high levels of inflammatory reaction persisted. Colonoscopy revealed discontinuous abnormal mucosal vascular patterns and ulcerations in the whole colon except for the rectum, and histological analyses of the intestine demonstrated transmural mucosal infiltration of inflammatory cells without epithelioid granuloma or amyloid deposition. Based on these findings, she was diagnosed with entero BD. Meanwhile, pedal edema appeared during her hospitalization. Urinalysis results were consistent with nephrotic syndrome, thus a renal biopsy was performed. Light microscopy showed no obvious glomerular and interstitial abnormalities, whereas electron microscopy revealed foot process effacement without immune complex deposition or fibrillary structure, compatible with minimal change disease (MCD). Only with conservative therapy, her proteinuria decreased, followed by a complete remission in 3 weeks from the onset of edema. The coincident episode of MCD was finally diagnosed as renal BD that paralleled disease activity to entero BD. She started adalimumab administration, resulting in the further improvement of diarrhea and inflammatory levels. Conclusions This is the first report to demonstrate MCD as renal involvement of BD along with the disease activity of entero BD.

It has been postulated that measles virus infection is associated with remission of idiopathic nephrotic syndrome (INS) in childhood. There are few reports on the correlation of INS remission with other infections. Previously, there have been two case reports suggesting an association between influenza B virus infection and the remission of INS. The patient was an 18-year-old Japanese woman. The onset of steroid-sensitive NS was at 9 years of age, and pathological diagnosis was minimal change nephrotic syndrome (MCNS). Until 10 months prior to visiting our hospital, the patient’s NS was in remission. The patient experienced fever, cough, and malaise and she was diagnosed with type B influenza by a local physician 4 days before visiting our hospital. The patient had vomiting and diarrhea 1 day prior to visiting our hospital. Her weight was 54.7 kg (+5.0 kg) and she had pitting edema of both lower legs. Her serum albumin level was 0.9 g/dL, proteinuria level was 8.73 g/gCr, and urine sediments showed 1–4 red blood cells per high-power field. She was diagnosed with relapse of NS. The level of proteinuria decreased to 0.03 g/gCr with rest alone on day 4 of admission, and a complete remission from NS was observed at approximately 2 weeks after the onset of influenza B infection. We report a rare case wherein spontaneous remission of NS occurred within a short period of 2 weeks after influenza B infection. It is clear that some immunity is involved in the pathogenesis of INS, but there are some cases in which infection improves NS and others in which it recurs.

Background. Recurrent spontaneous urticaria (RSU) is rare in children, thus, it is debilitating condition that always requires treatment. There are several limitations on the drugs use in children with RSU. Omalizumab is the effective medication for achieving the control of RSU, used in adolescents over 12 years of age.The aim of the study is to compare the efficiency of various approaches for RSU management in children.Methods. The three-year comparative study of patients with RSU aged from 1 to 17 years managed only with 2nd generation antihistamines in standard or increased doses for at least 3 months and patients managed in addition to standard treatment with omalizumab (300 mg once in 4 weeks subcutaneously) was conducted. The essential treatment outcomes are achieving control of the disease (UAS7 = 0) in 6 months and remission maintaining after 36 months of observation. Additional outcomes are decrease in the number of aggravations that required the use of glucocorticosteroids (GCS) during 3 years of followup; analysis of treatment outcomes of patients with severe urticaria in subgroups (including UAS7 levels).Results. The frequency of disease control (UAS7 = 0) by 6 months of therapy was significantly higher in patients of the omalizumab group — 76%, in the second group — 0%. The UAS7 in the omalizumab group was 0 (0; 1) points, in the group of patients on standard therapy — 13 (10; 16) points (p < 0.05). Remission was observed in 53% of patients (9 people) in the omalizumab group in 36 months, in the comparison group — in 32% (13 people), p = 0.129; UAS7 in the omalizumab group was 0 (0; 8.5) points, in the comparison group — 8 (0; 13) points, p = 0.076. The use of systemic GCS for acute treatment decreased during 3 years of follow-up: in the omalizumab group — from 41 to 5.9%, in the group on 2nd generation antihistamines — from 46 to 19% (p = 0.258). The result of subgroup analysis was similar.Conclusion. Adding omalizumab to standard RSU therapy makes it possible to achieve control of the disease reliably faster in most cases. There was gradual decrease in RSU activity in the group on standard therapy during the 3-year follow-up: spontaneous remission was mentioned in 32% of children within 3 years.

Abstract This report highlights the case of cystic adventitial disease of the left popliteal artery in a 45‐year‐old male patient. Imaging modalities confirmed the diagnosis and high resolution MRI found a cystic connection to the adjacent knee joint. The evolution was unusual with spontaneous regression of the symptoms.

Epstein-Barr virus mucocutaneous ulcer (EBVMCU) was first described by Dojcinov et al. in 2010 as a lymphoproliferative disorder presenting with a circumscribed cutaneous or mucosal ulcer in the setting of immunosuppression or immune senescence. Histologically, EBVMCUs show a polymorphous infiltrate of large B-cells with Hodgkin/Reed-Sternberg (HRS) - like cells intermixed with plasmacytoid cells and T cells which may be reminiscent of lymphomas. However, these lesions usually show an indolent disease course followed by spontaneous remission. In this article we present the case of a 33 year old immunocompetent male diagnosed with EBVMCU and also a review of oropharyngeal EBVMCUs.

Although novel therapies have improved the treatment outcome of patients, chronic lymphocytic leukaemia (CLL) is still considered incurable. Recently, a novel coronavirus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV2), causing coronavirus disease 2019 (Covid 19), emerged in late 2019, and it has posed a global health threat. In a limited number of cases, it has been shown that some lymphoma types spontaneously regress after SARS-CoV2 infection suggesting that the infection can trigger de immune system against the tumour cell. Cross-reactivity of pathogen-specific T cells with tumour antigens and natural killer cell activation can be the possible mechanism of this hypothesis.

Background: Idiopathic membranous nephropathy (IMN) is the most common form of primary nephrotic syndrome in adults. Antibodies against the M-type phospholipase A2 receptor (PLA2R-ab) are considered as diagnostic biomarkers of IMN. Objective: Here, we performed an updated meta-analysis to assess the diagnostic value of PLA2R-ab for clinical remission in IMN patients. Method: PubMed, Embase, and Cochrane databases were searched for relevant studies published before September 2022. Odds ratios and corresponding 95% confidence intervals were determined using a fixed or random effects model. The heterogeneity among studies was explored by subgroup analysis. Results: Sixteen studies involving 1,761 IMN participants were included. There were significant differences between PLA2R-ab (+) and PLA2R-ab (−) patients in terms of complete remission (CR) and spontaneous remission. The rates of partial remission (PR) and relapse were similar between the two groups. Patients with PLA2R-ab (−) were at a higher CR rate when treated with a calcineurin inhibitor or a treatment course for 3 months and 6 months, while the spontaneous remission rate was higher in PLA2R-ab seronegative patients from Asia. However, the CR and spontaneous remission rate only significantly declined in IMN patients with the highest titer, but not a middle titer, when compared to those with the lowest titer. Conclusion: In contrast with previous meta-analyses, our results verified that PLA2R-ab can likely predict CR and spontaneous remission in IMN patients, instead of PR and relapse. Race, immunosuppressive agents, and duration of treatment may affect the prognostic value of PLA2R-ab. Considering that the remission rate of IMN patients with a middle level of PLA2R-ab was not different from that of patients with the lowest level, a proper cut-off value of PLA2R-ab for prognosis should be clarified.

BackgroundHyperparathyroidism is a common cause of hypercalcemia; however, spontaneous remission after a hypercalcemic crisis caused by an intracystic hemorrhage of parathyroid adenomas is very rare. The question, then, is “What is the best treatment strategy for this type of case?”MethodA 47-year-old male patient with primary hyperparathyroidism and a hypercalcemic crisis is reported. Hypercalcemia was spontaneously relieved thereafter. Postoperative paraffin pathology results indicated an intracystic hemorrhage of bilateral parathyroid adenomas.ResultsAfter the case report, a literature review is also included to summarize the clinical features of this patient and to provide special reference for clinical diagnosis and treatment of similar cases.ConclusionsThe choice of surgical timing for such cases can be made based on the comprehensive consideration of clinical symptoms and changes in parathyroid function.

Chronic active Epstein–Barr virus infection (CAEBV) is relatively uncommon and can be associated with lymphoproliferative diseases (LPD's) of NK/T cell and B cell type. In the western world, the disease is less common, while it is found to be more prevalent in the East Asian, South, and Central American population. The presentation widely varies from skin rashes, fever, lymphadenopathy to major organ involvement and usually takes a fulminant course. Here, we describe a 70-year-old Indian male, who was diagnosed as CAEBV-related LPD with spontaneous regression with a brief review of the literature.

Abstract Diffuse large B‐cell lymphoma (DLBCL) is a heterogeneous group of lymphomas which require multiagent therapy for remission induction and are associated with relapse in more than 40% of patients. Spontaneous remission of diffuse large B‐cell lymphoma (DLBCL) is a rare occurrence.

Atopic dermatitis (AD) typically starts in infancy or early childhood, showing spontaneous remission in a subset of patients, while others develop lifelong disease. Despite an increased understanding of AD, factors guiding its natural course are only insufficiently elucidated. We thus performed suction blistering in skin of adult patients with stable, spontaneous remission from previous moderate-to-severe AD during childhood. Samples were compared to healthy controls without personal or familial history of atopy, and to chronic, active AD lesions. Skin cells and tissue fluid obtained were used for single-cell RNA sequencing and proteomic multiplex assays, respectively. We found overall cell composition and proteomic profiles of spontaneously healed AD to be comparable to healthy control skin, without upregulation of typical AD activity markers (e.g., IL13, S100As, and KRT16). Among all cell types in spontaneously healed AD, melanocytes harbored the largest numbers of differentially expressed genes in comparison to healthy controls, with upregulation of potentially anti-inflammatory markers such as PLA2G7. Conventional T-cells also showed increases in regulatory markers, and a general skewing toward a more Th1-like phenotype. By contrast, gene expression of regulatory T-cells and keratinocytes was essentially indistinguishable from healthy skin. Melanocytes and conventional T-cells might thus contribute a specific regulatory milieu in spontaneously healed AD skin.

Spontaneous remissions (SRs) in acute myeloid leukemia (AML) are infrequent, poorly documented and transient. Similarly, morphological and cytogenetic complete remissions (CR) under azacitidine treatment are scarce. We report a 71-year-old man with a secondary AML arising from essential thrombocythemia (ET), who developed an SR after discontinuation of azacitidine following a respiratory infection (four courses were administered). The distinctive feature of our case is the depth of the achieved CR, documented by next-generation sequencing (NGS) techniques. We also detected persistence of molecular lesions that might already have been present in the previous ET clone. Our patient relapsed 5 months after achieving CR. We conclude that our patient showed a spontaneous remission of his AML rather than an exquisite response to azacitidine. We hypothesize that the concurrent respiratory infection, or any other unknown trigger, might have activated his immune system forcing the leukemic stem cell to enter a quiescent state through a yet unexplained mechanism.

Idiopathic membranous nephropathy (IMN) is a pathological pattern of glomerular damage caused by an autoimmune response. Immune complex deposition, thickness of glomerular basement membrane, and changes in the podocyte morphology are responsible for the development of proteinuria, which is caused by the targeted binding of auto-antibodies to podocytes. Several auto-antigens have recently been identified in IMN, including M-type receptor for secretory phospholipase A2 (PLA2R1), thrombospondin type-1 domain-containing 7A (THSD7A), and neural epidermal growth factor-like 1 protein (NELL-1). The measurement of peripheral circulating antibodies has become an important clinical reference index. However, some clinical features of IMN remain elusive and need to be further investigated, such as the autoimmunity initiation, IgG4 predominance, spontaneous remission, and the unique glomerular lesion. As these unresolved issues are closely related to clinical practice, we have proposed a hypothetical pathogenesis model of IMN. Induced by environmental stimuli or other causes, the PLA2R1 antigen and/or THSD7A antigen exposed to extrarenal tissues, such as lungs, then produce the auto-antibodies that target and cause damage to the podocytes in circulation. In this review, we highlighted the potential association between environmental stimuli, immune activity, and glomerular lesions, the underlying basis for spontaneous immune and proteinuria remission.

Abstract Organizing pneumonia (OP) is a common interstitial lung disease, pathologically characterized by polypoid granulation tissue in the alveolar ducts and alveoli. In clinical practice, OP occasionally presents as non‐resolving pneumonia. The typical radiographic pattern of OP is characterized by dense consolidation with ground‐glass opacities. Diffuse micronodular pattern of OP (MNOP) is a rare radiographic manifestation that mimics non‐resolving bronchiolar diseases such as pulmonary tuberculosis or hypersensitivity pneumonitis. Steroid therapy is usually effective for MNOP; however, spontaneous remission in MNOP has never been reported. Herein, we report a case of a diffuse micronodular form of cryptogenic OP (COP) that was diagnosed via transbronchial biopsy (TBB) and resolved spontaneously within a few months. Our case highlights that MNOP may resolve spontaneously similar to other forms of OP, and mild cases may be under‐recognized. Furthermore, careful observation could be an option for managing MNOP with mild and non‐progressive symptoms.

Background: Langerhans Cell Histiocytosis (LCH) is a childhood disorder of histiocytes that is generally treated with systemic chemotherapy. Spontaneous resolution has been previously reported in Single System LCH (SS-LCH), which is less aggressive than multisystem disease. However, there are no clear guidelines on which patients can be safely spared from systemic chemotherapy. Here, we propose a risk stratification framework based on disease quiescence as determined by clinical and biochemical features of inflammation, to identify low risk patients who may be potentially spared from chemotherapy through a conservative “wait-and-see” approach.Methods: Retrospective analysis in a single institution was conducted in children with SS-LCH, comparing features of inflammation and outcomes of those who received chemotherapy vs. those with quiescent disease, who were managed conservatively.Results: Of 44 children with SS-LCH, only patients without risk-organ involvement were considered for conservative management. A “wait-and-see” approach was adopted for patients with quiescent disease as defined by clinical and biochemical evidence of disease activity. Following 2 weeks of watchful observation, decisions were made to either start treatment or continue conservative management. Based on data collected at diagnosis, patients with quiescent disease had a lower mean platelet count 339 × 109/L (95%C.I: 285–393) vs. 482 × 109/L (95% C.I: 420–544) p < 0.01, a lower mean white cell count 9.3 × 109/L (95%C.I: 7.5–11.1) vs. 13.1 × 109/L (95%C.I: 11–15.2) p < 0.01 and lower Erythrocyte-Sedimentation-Rate (ESR) 8.2 mm/h (95%C.I: 5.4–11) vs. 53.7 mm/h (95%C.I: 11–96.3) p = 0.04, suggesting that these are potential biochemical markers of disease activity. Other features of disease quiescence noted were rapid progression, functional disability, presence of a skull depression rather a lump and the lack of fever.Conclusions: Further studies are required to validate our proposed framework to determine disease activity in SS-LCH. Within the limits of this current analysis, it appears that low-risk patients with clinically and biochemically quiescent SS-LCH, may potentially be spared from chemotherapy with good long-term outcomes.

Immunoglobulin (Ig) A glomerulonephritis (GN) is a heterogeneous disease affected by various factors. Genetic and other factors “hit” DNA, causing IgA malformation and ultimately glomerular injury. We describe a rare case of crescentic IgA GN with sternoclavicular (SC) arthritis in a 75-year-old woman. Despite active IgA GN with cellular crescents, the patient achieved remission of IgA GN without glucocorticoid therapy after remission of SC arthritis was achieved. Considering the patient’s clinical course, this case suggested a relationship between IgA GN and SC arthritis.

According to the literature, spontaneous remission of aggressive lymphomas is extremely rare; gastric non-Hodgkin lymphomas, such as mucosa-associated lymphoid tissue lymphomas, often regress due to Helicobacter pylori treatment or no progression, even in a watch-and-wait strategy. Although spontaneous remission of diffuse large B cell lymphomas in the stomach was very rarely reported, the follow-up periods of the cases of spontaneous remission are within 2 years and most cases are likely to relapse after the first remission. Here, we report that a diffuse large B cell lymphoma in the stomach showed spontaneous remission within 2 months after the initial diagnosis and the remission is still continuing for 10 years without any specific treatments against this aggressive lymphoma.

Abstract Background While the majority of adult-onset minimal change nephrotic syndrome (MCNS) is a primary or an idiopathic form of disease, it can also occur as a secondary form. Reports on the spontaneous remission of MCNS are rare since the condition is typically treated with corticosteroids. We herein describe the spontaneous remission of adult-onset MCNS in a patient who developed nephrotic syndrome after type B influenza infection. Case presentation A 50-year-old woman experienced fever, cough, malaise, and low back pain, which had persisted for 6 days before she presented to our hospital, and edema of the face and limbs, which had persisted for 5 days before her presentation. She was diagnosed with type B influenza infection and later exhibited an exacerbation of facial edema, decreased urine output, and a high level of proteinuria. She was referred to our department after the diagnosis of nephrotic syndrome. On admission, her proteinuria level was 20.88 g/gCr and her selectivity index value was 0.13. The examination of a kidney biopsy specimen obtained on the fourth day of hospitalization under a light microscope revealed minor abnormalities. An immunofluorescence showed only nonspecific granular IgM deposits in the mesangium. Electron microscopy showed extensive foot process effacement without any immune complex deposits. Based on these findings, the patient was diagnosed with MCNS. After admission, the proteinuria decreased to 0.06 g/gCr with rest and sodium restriction (6 g/day) alone; a complete remission from nephrotic syndrome was observed at approximately 2 weeks after the onset of symptoms. There have been no signs of recurrence of nephrotic syndrome in the one years since. Conclusion We experienced a rare case in which spontaneous remission of MCNS occurred within a short period of 2 weeks after influenza B infection. When patients present with nephrotic syndrome after an infection, it is necessary to consider MCNS in the differential diagnosis, which also includes post-infectious glomerulonephritis and the acute exacerbation of IgA nephropathy.

Abstract Introduction Chronic subdural hematoma (CSH) is a hemorrhagic brain injury that persists for more than 21 days after its initial formation. The incidence is predominantly among the elderly population (> 65 years), and varies from 58 to 74/100,000 inhabitants. Spontaneous resolution is considered variable; in the literature series, it is

Acute myeloid leukemia (AML) is defined by the presence of ≥ 20% myeloblasts in the blood or bone marrow. Spontaneous remission (SR) of AML is a rare event, with few cases described in the literature. SR is generally associated with recovery from an infectious or immunologic process, and more recently possibly with clonal hematopoiesis. We review the literature and assess the trends associated with SR, and report a new case of a 58-year-old man with a morphologic diagnosis of AML associated with a severe gastrointestinal (GI) tract infection. The patient had an NF1 variant that was previously unreported in AML as the only clonal abnormality. After treatment of the infection, the increased blast population subsided with no leukemia-directed therapy, and the patient has remained in a continuous, spontaneous complete remission for > 2 years.

Natural killer (NK) cell lymphoproliferative disorders are uncommon and the Epstein-Barr virus (EBV) plays an important aetiological role in their pathogenesis. We report a 20-year-old male with a chronic active EBV infection associated with a NK cell lymphoproliferative disorder which had an unusual indolent course. He presented to the Sultan Qaboos University Hospital in Muscat, Oman, in December 2011 with a history of intermittent fever and coughing. Examinations revealed generalised lymphadenopathy, hepatosplenomegaly, leukocytosis, transaminitis, diffuse bilateral lung infiltrates and bone marrow lymphocyte involvement. A polymerase chain reaction (PCR) test revealed a high EBV viral load in the peripheral blood cells. The patient received a course of piperacillin-tazobactam for Klebsiella pneumoniae, but no active treatment for the lymphoproliferative disorder. However, his lymphocyte count, serum lactate dehydrogenase and liver enzymes dropped spontaneously. In addition, EBV PCR copies fluctuated and then decreased significantly. He remained clinically asymptomatic over the following four years.

Diffuse large B-cell lymphoma (DLBCL) is the most common type of lymphoma. Although DLBCL can be cured in more than half of all patients, up to 50% of patients become refractory to initial treatment or relapse after complete remission. We present a case of complete spontaneous remission of some tumors and concomitant newly developed tumors observed in a patient with relapsed DLBCL. Spontaneous remission of lymphoma without treatment is a rare phenomenon and can occur at baseline as well as in relapsed DLBCL. However, most patients who initially experience spontaneous remission later develop relapse. Thus, careful follow-up is required, and fluorine-18-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) allows monitoring of multiple lesions.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired syndrome characterized by intravascular hemolysis, thrombosis, and bone marrow failure. The disease is caused by a mutation in the PIG-A gene that leads to the lack of glycosylphosphatidylinositol-anchored complement regulatory molecules CD55 and CD59 on affected blood cell surfaces. In previous studies, spontaneous clinical remissions have been described. The disease manifestations are very heterogeneous, and we wanted to examine if true remissions and disappearance of the clone occur. In a follow-up of a nation-wide cohort of 106 Finnish patients with a PNH clone, we found six cases, where the clone disappeared or was clearly diminished. Two of the patients subsequently developed leukemia, while the other four are healthy and in clinical remission. According to our data, spontaneous remissions are not as frequent as described earlier. Since the disappearance of the PNH cell clone may indicate either a favorable or a poor outcome—remission or malignancy—careful clinical monitoring in PNH is mandatory. Nevertheless, true remissions occur, and further studies are needed to understand the immunological background of this phenomenon and to obtain a better understanding of the natural history of the disease.

PurposeThe purpose of this study was to determine the frequency of CD4+CD25+FoxP3+ regulatory T cells (Treg) in the peripheral blood of patients with childhood chronic immune thrombocytopenic purpura (ITP) exhibiting thrombocytopenia and spontaneous remission. The findings of this study indicate the possibility of predicting spontaneous recovery and pathogenesis of childhood chronic ITP.MethodsEleven children with chronic ITP (seven thrombocytopenic and four spontaneous remission cases; mean age, 8.8 years; range, 1.7-14.9 years) were enrolled in this study. Five healthy children and eight healthy adults were included as controls. The frequency of Treg was evaluated by flow cytometry in the peripheral blood.ResultsIn this study, four patients (36%) achieved spontaneous remission within 2.8 years (mean year; range, 1.0-4.4 years). The frequency of Treg was significantly lower in patients with persisting thrombocytopenia (0.13%±0.09%, P

A apresentação clínica da sarcoidose e diversa e em mais de 90% dos casos existe envolvimento pulmonar. Os achados radiológicos mais frequentes aquando do diagnostico são a presença de adenopatias hilares e infiltrados pulmonares. Os autores apresentam o caso de uma doente jovem cuja radiografia torácica apresentava múltiplos nódulos bilaterais. A biopsia cirúrgica revelou a presença de granulomas nao necrotizantes com células gigantes multinucleadas, compatível com sarcoidose. Apesar de se tratar de uma sarcoidose pulmonar estádio III, a doente apresentava -se assintomática, o estudo funcional ventilatorio era normal e não havia sinais de envolvimento extratoracico, pelo que foi decidido não iniciar tratamento. Houve remissão espontânea das lesões, comprovada em tomografia computorizada de alta resolução após um ano.The clinical presentation of sarcoidosis is diverse and in over 90% of patients there is pulmonary involvement. The most common features of the radiographic findings at the time of diagnosis are bilateral hilar lymphadenopathy and pulmonary infiltration. The authors report the case of a young female patient who presented with multiple bilateral nodular shadows on chest radiograph. Surgical biopsy revealed non-necrotizing granulomas with occasional multinucleated giant cells compatible with sarcoidosis. Al-though this was a case of stage III pulmonary disease, the patient was asymptomatic, lung function tests were normal and there were no signs of extrathoracic involvement. Spontaneous remission occurred without treatment as shown on high resolution CT scan follow-up, one year later.

A recanalização espontânea tardia da carótida interna é um evento incomum e pouco estudado. Os autores relatam o caso de paciente de 73 anos, masculino, hipertenso, com antecedente de acidente vascular cerebral há 3 anos, com seqüela motora e sensitiva em membro superior direito, apresentando ao mapeamento dúplex e arteriografia oclusão total da carótida interna. Evoluiu após 2 anos com novos episódios de ataques isquêmicos transitórios de repetição. Submetido a novos exames de imagem para avaliação da circulação cerebral extra e intracraniana, evidenciou-se recanalização do vaso, com estenose severa. Foi realizada endarterectomia de carótida esquerda, sem intercorrências. Paciente evoluiu sem novos episódios após 1 mês de seguimento. Considerando a raridade do caso e a parca literatura sobre recanalização tardia de carótida, passível de reparo cirúrgico, optamos pela apresentação do caso enfocando a importância do acompanhamento de oclusões carotídeas.Late spontaneous recanalization of internal carotid artery is an unusual event that has received little attention. The authors report a case of a 73-year-old male patient, hypertensive, with previous history of cerebral vascular accident 3 years ago, with sensorimotor sequela in the right upper limb. Duplex scanning and arteriography showed total occlusion of the internal carotid artery. The patient progressed after 2 years with new episodes of recurrent transient ischemic attacks. When submitted to new imaging examinations to assess extra- and intracranial circulation, vessel recanalization was demonstrated, with severe stenosis. Left carotid endarterectomy was uneventfully performed. The patient progressed without new episodes after a 1-month follow-up. Considering the case rarity and the lack of literature on late carotid artery recanalization that can be surgically repaired, we decided to present this case focusing on the importance of following carotid artery occlusions.

Blueberry muffin baby is a rare neonatal skin disorder. Causes for the generalized hemorrhagic purpuric eruptions include congenital infections, hemolysis, and tumors. We report a 2.5-month-old female baby with a blueberry muffin appearance, respiratory distress, and decreased activity and appetite. Skin biopsy showed diffuse infiltrates of myeloperoxidase- and lysozyme-positive blast-like cells in dermis and superficial subcutis. Bone marrow study confirmed the diagnosis of acute monocytic leukemia with leukemia cutis. The skin nodules regressed spontaneously without chemotherapy over several days, and the peripheral blood cell counts normalized. This spontaneous remission lasted for 2 months. Spontaneous remission of infantile leukemia is rare, and its mechanism remains unknown. Although overt leukemia relapsed in some of these patients, a delay in chemotherapy spared these infants of the toxic effects of treatment.

A community-based cohort of 77 children with new-onset temporal lobe epilepsy (TLE) were followed prospectively and reviewed at 7 and 14 years after seizure onset, and clinical, EEG, and neuroimaging findings and seizure outcome are reported from the Royal Children's Hospital and University of Melbourne, Australia, and Starship Children's Hospital, Auckland, New Zealand.