Solitary Langerhans cell histiocytosis of the sternum in a 21-year-old woman.

Children are more likely to develop Langerhans cell histiocytosis (LCH), a rare disorder with an unknown cause. LCH often invades skeletal systems, while it has occasionally been seen in the sternum or ribs. The best course of treatment for single-site, skeletal LCH is yet unknown. We present an instance of sternal LCH with adult onset. By fusing and reconstructing chest computed tomography, it was possible to determine the extent of surrounding soft tissue invasion. Because LCH is so uncommon, it could be challenging to recall when we see a sternal lesion. Adult Patients who arrive with anterior chest discomfort and an osteolytic sternal lesion should include LCH on their differential diagnosis list.

Severe Hepatotoxicity in Mushroom Poisoning by Lepiota brunneoincarnata from Complete Recovery to Liver Transplantation: A Case Series with Review on Liver Function Tests and Liver Histopathology.

Background: In spite of the scientific evidence supporting health advantages of mushrooms, some of them are seriously poisonous. The clinical picture of mushroom intoxication ranges from minor gastrointestinal symptoms to organ failure, such as liver failure and death. Method: We provided demographics, clinicopathological characteristics, applied treatments, and outcomes of mushroom poisoning by Lepiota species in a series of 18 cases that were referred from Kermanshah and Lorestan provinces to Abu-Ali-Sina Hospital, Shiraz, Iran. Clinical and paraclinical data were collected by taking history and reviewing of medical documents. Pathologic findings were extracted through a review of hematoxylin and eosin pathologic slides. Results: The patients were between the ages of 18 and 67 years, composed of ten females and eight males. The most frequent clinical manifestations were nausea and vomiting followed by abdominal pain. Four cases presented decreased consciousness on admission. One of them passed away. Three other cases underwent liver transplantation, two of them died after transplantation, and one fully recovered without any major issues. All instances had elevated ALT levels, which ranged from 44 to 9,140 IU/L (mean: 3259 ± 2476), with most of them also having concurrent AST elevations (mean: 1,361 ± 1,532). Only few patients had modest elevations in alkaline phosphatase. Total and direct bilirubin elevations up to 47.6 and 24 mg/dL, respectively, were found in most cases. Decreased total protein and albumin concentrations and increased BUN and creatinine levels were observed in some patients. In addition, some instances revealed increased LDH, increased WBC, decreased hemoglobin, and decreased platelet count. Most patients had increased prothrombin time; hematuria and positive stool occult blood were observed in few patients. Histopathologic examination of three explanted livers revealed massive necrosis with moderate to severe macrovesicular steatosis, significant ductular reaction, and parenchymal inflammation. Other patients followed a recovery process with a considerable drop in liver enzymes, especially ALT, during hospitalization utilizing conservative treatment. They had no liver problems or relevant issues after a two-year follow-up. Conclusion: In our study, highly elevated liver enzymes with a significantly high ALT/AST ratio were observed in cases of mushroom poisoning by Lepiota species, leading to fulminant liver failure and death in some cases. These laboratory findings were correlated with liver necrosis and macrovesicular steatosis in explanted livers.

Comparing Jaffe and Enzymatic Methods for Creatinine Measurement at Various Icterus Levels and Their Impacts on Liver Transplant Allocation.

The Model for End-Stage Liver Disease (MELD) scoring system is used to prioritize liver transplantations and assess disease severity. This includes the international normalized ratio (INR), creatinine, and total bilirubin. Since there are several ways to measure creatinine, MELD scores can produce inconsistent results. The objectives of this study were to define a valid cut-off for bilirubin interference in creatinine measurement and to assess the effects of various icteric levels on creatinine measurement and liver transplant allocation. A total of 400 serum samples were categorized into four groups based on their icteric indices and total bilirubin levels, including non-, mild, moderate, and severe icteric samples. Both chemical Jaffe and enzymatic techniques were used to determine the creatinine levels in all four groups, and the findings were compared. In parallel, serum samples from 83 liver transplant candidate patients were divided into three groups depending on their bilirubin levels and then similarly evaluated and interpreted. The MELD scores were then computed for each group and compared. In icteric samples, the enzymatic method produced higher results for the creatinine concentrations than the Jaffe method did, and the mean creatinine difference rose from 0.08 in nonicteric group to 1.95 in groups with severe icterus. In addition, the enzymatic approach yielded higher findings for creatinine and subsequently for MELD scores in patients who were liver transplant candidates. When the bilirubin concentration was above the 4 mg/dL threshold, there were differences between the approaches for both the creatinine and the MELD score (p values: 0.0001 and 0.027, respectively). The chemical Jaffe is a readily available and considerably cost-effective method for measuring creatinine. However, it is influenced by a variety of known and unknown interfering substances, and it should be applied cautiously when working with icteric samples. Alternate techniques such as the enzymatic method should be considered when the bilirubin level exceeds 4 mg/dL. Though this cut-off is instrument and kit-dependent, each laboratory is advised to have its cut-off for bilirubin interference.

Sclerosing angiomatoid nodular transformation of the spleen in a child with anemia: a case report and review of the literature.

BACKGROUND: Sclerosing angiomatoid nodular transformation of the spleen is a relatively rare benign vascular lesion in both adult and pediatric age groups with unclear etiopathogenesis and variable clinical presentations. Many benign and also malignant splenic masses could mimic sclerosing angiomatoid nodular transformation, both clinically and radiologically. Herein, we report our experience with a case of sclerosing angiomatoid nodular transformation in a 3-year-old girl. CASE REPORT: A 3-year-old Iranian girl presented with abdominal pain, back pain, and constipation for 2 weeks. She was being followed up by a pediatrician due to her short stature and persistent anemia. Physical examination showed stable vital signs, short stature, pallor, and a puffy face. Laboratory evaluation showed normochromic normocytic anemia with a normal reticulocyte count, ferritin, and hemoglobin electrophoresis. Radiologic assessments revealed a hypoechoic lesion in the spleen with high vascularity, clinically suspected to be lymphoma. She was operated on, and after partial splenectomy, pathologic evaluation of the spleen showed a solitary, well-demarcated, and unencapsulated dark mass. Microscopic examination revealed micronodular appearance composed of irregular-shaped vascular spaces lined by plump endothelial cells and surrounded by concentric collagen fibers, features in keeping with sclerosing angiomatoid nodular transformation. The patient's anemia was resolved after surgery, and no clinical or radiologic deficits were noted during the 10-month follow-up visits. CONCLUSION: Although sclerosing angiomatoid nodular transformation is exceedingly rare in children, it should be considered a differential diagnosis in pediatric splenic neoplasms with concurrent hematologic manifestations, such as anemia.

A case report: Bilateral reconstruction of C2 lateral masses with expandable titanium cages following axis (C2) solitary plasmacytoma resection with 2-year follow-up.

Background: Solitary plasmacytoma (SP) caused the collapse/destruction of the C2 vertebral body in a 78-year-old male. To provide sufficient posterior stabilization, the patient warranted lateral mass fusion to supplement the bilateral pedicle/screw rod instrumentation. Case Description: A 78-year-old male presented with neck pain alone. X-rays, computed tomography, and magnetic resonance studies documented C2 vertebral collapse with the complete destruction of both lateral masses. The surgery required a laminectomy (i.e., bilateral lateral mass resection), plus placement of bilateral expandable titanium cages from C1 to C3 to supplement the screw/rod occipitocervical (O-C4) fixation. Adjuvant chemotherapy and radiotherapy were also administered. Two years later, the patient remained neurologically intact and radiographically had no evidence of tumor recurrence. Conclusion: In patients with vertebral plasmacytomas and bilateral lateral mass destruction, posterior occipital-cervical C4 rod/screw fusions may warrant the additional bilateral placement of titanium expandable lateral mass cages from C1 to C3.

Case report: A case of renal arcuate vein thrombosis successfully treated with direct oral anticoagulants.

A rare case of a 35 years old woman presented with renal arcuate vein thrombosis (RAVT) and acute kidney injury (AKI) following upper respiratory tract symptoms and toxic substance ingestion. Histopathological evaluation of the patient's kidney tissue indicated a rare venous thrombosis in the renal arcuate veins. Anticoagulation with Apixaban, a direct oral anticoagulant (DOAC), was commenced, and the patient's symptoms resolved during the hospital stay. Hitherto, a limited number of studies have shown the concurrent presentation of RAVT and overt AKI in patients following ingestion of nephrotoxic agents. Further studies are necessary to elucidate the etiology, clinical presentation, and treatment of RAVT. We suggest that Apixaban be studied as a suitable alternative to conventionally used anti-coagulants such as Warfarin in patients who lack access to optimal health care facilities.

Uninvited guest, Balantidium coli in urine in a patient with bladder cancer: A case report and review of the literature.

Key Clinical Message: In contrast to intestinal balantidiasis, which is widespread throughout the world, urinary balantidiasis is uncommon. It often affects people with underlying diseases, and acute infections may be fatal. Even though urine is not typical for this parasite, specific morphologic characteristics can aid in accurate diagnosis. Abstract: Balantidium coli is a ciliated protozoan which can infect intestinal system. Urinary balantidiasis is an extremely rare infection that may cause serious issues in patients with underlying diseases. Herein, we present a case of urinary balantidiasis in a patient with bladder cancer.

Splenic Lymphangioma Mimicking Lymphomatous Involvement: A Case Report with Review of the Literature.

Lymphangioma is a benign malformation of lymphatic vessels usually found in the head and neck areas or axilla. They may involve visceral organs with a lower percentage. Splenic lymphangioma is a rare tumor. This disease is often seen in children but may be diagnosed incidentally in adults. Most patients are asymptomatic, but in large and multifocal lesions, the patient may have some nonspecific symptoms such as abdominal pain, abdominal distention, nausea, vomiting, and loss of appetite. Physical examination may show no specific findings or detect palpable masses. The preoperative diagnosis of splenic lymphangioma is challenging. Histopathological evaluation and sometimes immunohistochemistry tests can result in a definitive diagnosis. In this study, we present an 18-year-old man, with Burkitt's lymphoma who underwent laparotomy and total splenectomy as a result of cystic lesions discovered accidentally during imaging with the final diagnosis of splenic lymphangioma after histopathological evaluation.

Critical value in surgical pathology: evaluating the current status in a multicenter study.

BACKGROUND: The concept of critical value is not evident in surgical pathology, and there is no established protocol for determining, reporting, and documenting these results. MATERIALS AND METHODS: A questionnaire was designed regarding critical value in surgical pathology, and all pathologists and some clinicians from five laboratories were asked to participate through an invitation link. The most important items were selected, and all pathologists were instructed to follow a standard operating procedure to deal with critical results for a year. RESULTS: A total of 43 pathologists and 44 non-pathologists participated in the study. Some critical or unexpected items were selected. Most participants agreed that the optimal time to announce critical reports is within 24 h of establishing the final diagnosis, and a phone call was the most dependable communication option. In addition, the most qualified recipients were the attending physicians. Therefore, a written policy was implemented for a year. One hundred seventy-seven critical or unexpected cases (0.5%) were detected. Mucormycosis and cytomegalovirus (CMV) were the most frequent critical cases. CONCLUSION: There are no set criteria for critical items or the reporting process in surgical pathology. It is possible to establish more uniform norms for reporting these cases by boosting pertinent research efforts and recruiting more pathologists and physicians. Additionally, it is advised that each medical facility compile its own unique critical or unexpected diagnosis list.

Do Serological Tests Eliminate the Need for Endoscopic Biopsy for the Diagnosis of Symptomatic Patients with Celiac Disease? A Retrospective Study with Review of Literature.

Background: Celiac disease is one of the most common genetic allergies worldwide. The prevalence of celiac disease in Iran is similar to or even higher than the global prevalence. Celiac disease is a chronic inflammatory disease that affects the small intestine. Affected patients are allergic to gluten protein that exists in some grains, such as wheat and barley. Methods: Serological endomysial IgA antibody (EMA-AB) and tissue transglutaminase IgA antibody (TTG-IgA) tests were performed on 114 patients aged the ages of 0-18 years with histopathological findings of celiac disease. The results of these tests were compared to the results of the histopathological study of the duodenal biopsy. Results: Based on the receiver operating characteristic (ROC) curve and a calculation of the TTG-IgA test's sensitivity and specificity, the best diagnostic limit for the TTG-IgA test is 144, which has the best sensitivity and specificity. At this value (cut-off), the test's sensitivity was 62%, and the specificity was 93.7%. For the endomysial test, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were 80%, 93%, 90%, and 75%, respectively. Conclusion: The diagnostic accuracy of the endomysial test is better than that of the TTG-IgA test in general for diagnosing patients with celiac disease. In the TTG-IgA test, false-positive cases are high due to a cut-off of 20, reducing the test's specificity. In these false-positive cases, the endomysial test helps in better diagnosis.

Diagnostic Accuracy of Direct Immunofluorescence Test on Paraffin-Embedded Blocks in Comparison with Frozen Section Blocks in Renal Biopsies.

Background: In several published research, the evaluation of renal disorders using immunofluorescence on formalin-fixed, paraffin-embedded (FFPE) tissue sections versus immunofluorescence on frozen sections was compared. Each technique's accuracy varies greatly. This study's objective was to assess IF-P as a potential replacement for IF-F in the diagnosis of renal biopsy specimens. Materials and Methods: To show immunoglobulin IgA, IgG, IgM, and C3 immune deposits, proteinase K digestion of paraffin-embedded renal biopsy was standardized and used in 51 renal biopsies. Sensitivity, specificity, false-positive, and false-negative values were calculated. Results: IF-P showed a sensitivity of 93.1%, 76.9%, 63.6%, and 33.3%, and a specificity of 100%, 97.3%, 95%, and 100% for IgG, IgA, IgM, and C3, respectively. Compared to cases that had both routine IF and IF-P, 50 of 51 showed either the same amount of staining for the diagnostic immunoglobulin/complement or a small amount of difference. In most of the cases (49 of 51), diagnostic findings were found. Conclusion: IF-P is a sensitive and precise approach for assessing immune deposits in renal tissue biopsies. We come to the conclusion that IF-P serves as a beneficial salvage immunohistochemistry method for renal biopsies that do not contain enough cortical tissue for IF-F.

Evaluation of the Iranian panel reactive antibody calculator and potential usefulness: A retrospective study.

BACKGROUND AND OBJECTIVES: There are several cPRA websites based on large enough samples in Eurotransplant, the United Network for Organ Sharing (UNOS), and the Canadian Transplant Registry (CTR). On the other hand, those calculators can differ based on the ethnicity to which they are applied. We developed the Iranian PRA calculator and compared it with UNOS and CTR calculators. METHODS: The allele and haplotype frequencies of the Iranian donor pool were estimated using the HLA typing of 523 deceased Iranian kidney donors. The Organ Procurement and Transplantation Network formula was used to generate cPRA (cPRA frequency). We also used a computer script to compare the undesirable antigens of patients with the human leukocyte antigen (HLA) phenotype of donors (cPRA filtering). A total of 100 anti-HLA antibody profiles were determined in 100 sensitized individuals on the waiting list, and cPRA was estimated using various PRA calculators. RESULTS: Variable allelic frequencies were obtained from population heterogeneity in each calculator's donor panel. However, no significant changes in cPRA were identified between the Iranian calculator, UNOS, and the Canadian online calculators. Lin's concordance correlation coefficient of .98 showed that cPRA (freq) and cPRA (filter) values had almost perfect agreement. INTERPRETATION AND CONCLUSION: The cPRA values from the Iranian calculator are comparable to those from UNOS and CTR calculators. The donor filtering method was more useful because of factors like cost and flexibility. It also makes it easier to update cPRA on a regular basis.

Glomerulopathy in patients with dermatomyositis in early active disease: clinical, pathological and capillaroscopic manifestations, and response to treatment.

Introduction: Idiopathic inflammatory myopathies (IIMs) are a group of systemic connective tissue diseases that present with muscular and extra-muscular manifestations. There are few reports on kidney involvement, especially in dermatomyositis (DM) patients. We evaluated the clinical, laboratory, capillaroscopy, and kidney pathology of patients with DM, who presented with proteinuria during the first year, and followed them for response to treatment. Material and methods: We evaluated 205 patients with proximal muscle weakness or high muscle enzymes, who referred to the nailfold capillaroscopy clinic from April 2010 to October 2021. Seventy-four patients fulfilled the New 2017 EULAR/ACR Classification Criteria for adult and juvenile IM with probability of ≥ 90% for DM with duration of ≤ 12 months and proteinuria > 350 mg/24 hours. All manifestations of patients with glomerulopathy and their kidney biopsies were reviewed, and they were followed for their treatment response. Results: From 74 patients with DM, 52 female and 22 male, median age 37 (19-65) years, and disease duration of median 4.5 (1-12) months, 2 (2.7%) patients (25- and 28-year-old male) had proteinuria. Their kidney biopsy showed mesangioproliferative glomerulonephritis (GN). There was no case of acute or chronic kidney damage or rhabdomyolysis. Both had high disease activity, high erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), abnormal capillaroscopy, and high anti-Ro positivity with good early response of their kidney function, muscle weakness, and laboratory tests after immunosuppressive treatment for 3-6 months. One patient had capillaroscopy follow-up, and all abnormalities were resolved in 8 fingers. One patient, due to poor follow-up, after 8 months had recurrence of his disease. Conclusions: We found mesangioproliferative GN as a rare extra-muscular manifestation in patients with DM in the active and early phase of the disease. Full immunosuppressive treatment showed early complete recovery in these patients.

A component could not be larger than the whole, polyclonal immunoglobulin interference in conjugated/direct bilirubin assay and elimination: A case report.

Analytical interferences (hemolysis, icterus, paraproteinemia, and lipemia) are of great concern in laboratory tests. In our case report, the conjugated bilirubin result of a patient was significantly higher than the total bilirubin. We recommend being more cautious with samples that produce indistinguishable results and attempting to resolve the issues before releasing them.

And the Oscar Goes to Peripheral Blood Film for the Detection of Lead Poisoning in a Complicated Toxic Patient: A Case Report with a Review of Laboratory Clues.

BACKGROUND: Peripheral blood smear examination is an invaluable laboratory test, which provides the complete hematologic and/or nonhematologic picture of a case. In addition to verifying the results of automated cell counters, it has the potential to identify some pathologic and morphologic changes that remain hidden using the cell counters alone. Case Presentation. A 40-year-old man with a three-year history of alcohol intake and marijuana abuse presented with severe lower extremities of the bone and abdominal pain. Physical examination showed high blood pressure, high pulse rate, and abdominal tenderness. He underwent extensive laboratory and imaging tests, and cholecystectomy and bone marrow studies were associated with no definite diagnosis. Right after all these invasive, expensive, and time-consuming investigations during a month, finding coarse basophilic stippling in the red blood cells in the peripheral blood smear by an expert led to the final diagnosis. Elevated blood lead level and the presence of ring sideroblasts in the bone marrow study confirmed the diagnosis of lead poisoning, and the patient responded well to chelator therapy in a short period. CONCLUSION: This case clearly showed the value of peripheral blood smear review and its impact on patient care. In order not to lose the cases, laboratories are recommended to design their own policy for peripheral blood smear review. The peripheral blood smear is the fastest, simplest, and most available screening test, which can prevent many misdiagnoses and malpractices. It provides rich morphological information, among which basophilic stippling is highly suggestive of lead poisoning.

Comparison of Point-of-Care and Highly Sensitive Laboratory Troponin Testing in Patients Suspicious of Acute Myocardial Infarction and Its Efficacy in Clinical Outcome.

BACKGROUND: The use of high-sensitivity troponin (hs-cTnI) assays is recommended in current guidelines for managing patients with acute coronary syndrome (ACS) symptoms. However, point-of-care (POC) assays are frequently used in emergency departments (EDs) to reduce turnaround time and length of stay. This study aimed to compare the results of POC-cTnI testing with those of the gold standard, automated central laboratory testing of troponin (i.e., hs-cTnI). The primary and secondary outcomes were the diagnostic performance of POC-cTnI in diagnosing acute myocardial infarction (AMI) and major adverse cardiovascular events (MACE) during 30 days, respectively. MATERIALS AND METHODS: In this diagnostic accuracy study, 136 patients with suspected ACS who were referred or admitted to the Al Zahra Hospital, Shiraz, Iran, were included between March (2020) and July (2020). For the diagnosis of AMI, central laboratory cTnI levels were assessed at the time of presentation (0 hour) and reassessed at least 3 hours later. The POC-cTnI was measured at 0 hour in all patients and at 3 hours if a patient was diagnosed with AMI but had a 0-hour negative result for the POC-cTnI assay. Additionally, the 30-day follow-up period for these participants began on the day of the initial presentation to assess MACE. RESULTS: Out of 180 patients, 136 patients (median age of 59.5 years; 57.5% male) were left for the qualitative POC-cTnI and hs-cTnI assays. In 86 (63.24%) subjects, hs-cTnI was positive (either initial or serial); however, AMI was diagnosed in 85 patients according to positivity of troponin by hs-cTnI and clinical signs and symptoms, which were diagnosed by a cardiologist. The sensitivity, specificity, and negative predictive value of 0-hour POC-cTnI were observed to be 91.76% (95% CI: 83.77-96.62%), 98.04% (95% CI: 89.55-99.95%), and 87.72% (95% CI: 77.82-93.56%), respectively. Moreover, considering both the 0-hour and 3-hour POC-cTnI, all AMI cases were correctly identified, yielding a perfect test performance result. None of the 50 patients with negative cTnI results (by 0-hour and 3-hour POC-cTnI and hs-cTnI) experienced at least one MACE. CONCLUSION: In this small sample-size study, a new qualitative POC-cTnI assay was statistically equal to a hs-cTnI assay in terms of diagnostic accuracy for AMI or MACE in patients with suspected myocardial infarction. The POC-cTnI was observed to be acceptable for the identification of AMI and prediction of MACE in the ED environment.

Two cases of cardiac hemangioma in different anatomical locations presenting with chest pain and palpitation.

Cardiac hemangiomas are benign, slow-growing tumors among the most common soft tissue tumors in the human body. Surgical resection is highly recommended for cardiac hemangiomas in symptomatic patients. We report our cases with a review of the literature on cardiac hemangiomas.

Off Clamping Laparoscopic Resection of An Extreme Rare Renal Mesenchymal Tumor: An Angiomyolipoma of Renal Capsule.

Renal mesenchymal tumors are described as neoplasms with vascular, fibrous, and adipose tissues. The renal lipoma is an extremely rare renal mesenchymal tumor, typically originating from renal capsule and it is usually presented as well circumscribed homogenous fat containing mass. Angiomyolipoma (AML) is the most common benign mesenchymal renal tumor which is composed of mature epithelioid cells. The renal AML usually presented as exophytic, non-infiltrative, and fat contain tumor. The well differentiated renal retroperitoneal liposarcoma and lipoma seem to be misdiagnosed by exophytic renal angiomyolipoma but the renal AML usually arises from renal parenchyma with characteristic images. A 37-year-old woman came to our clinic with rapid growth renal mass and pain. The spiral abdominopelvic computed tomography scan (CT-scan) showed well-circumscribed hypoheterodense fat-containing mass near to middle pole of the right kidney with minimal fat stranding without neovascularity and cortical defect. The Patient underwent off-clamping laparoscopic resection of renal mass with pre-operative impression: liposarcoma versus lipoma of the kidney. The cross-section of the surgical specimen revealed irregular lobulated fatty tissue with hemorrhagic streaks. Definite diagnosis was made by immunohistochemistry study. Spindle cells and epithelioid cells are diffusely and strongly positive for α-smooth muscle actin. The perivascular cells and epithelioid cells are positive for HMB-45 and Melanin. The immunostaining pattern was compatible with angiomyolipoma that originated from renal capsule. In our experience, a rapid growing mass that is accompanied by pain draws the attention to malignant process. The renal AML rarely arises from renal capsule without characteristic images so having high doubt may lead to proper pre-operative diagnosis.