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Assessment of Hepatic Profile in Acquired Aplastic Anemia: An Experience From Pakistan.

INTRODUCTION: Aplastic anemia (AA) is characterized by pancytopenia and hypocellular marrow in the absence of an abnormal infiltrate or increase in reticulin fibrosis. The diagnosis of AA is challenging at times due to decreased cellularity and overlapping morphological features with other bone marrow failure syndromes. Hepatitis-associated aplastic anemia (HAAA) is a rare variant in which patients typically present with jaundice and hepatitis followed by pancytopenia almost within 6 months. Post-hepatitis AA accounts for approximately 1-5% of cases, and invariably such cases are negative for …

Prevalence of silent celiac disease in patients with autoimmune thyroiditis from Northern Sardinia.

Celiac disease (CD) is frequently associated with other autoimmune diseases such as Type 1 diabetes mellitus, autoimmune thyroiditis (AT), and Addison's disease. The frequency of these associations varies with the populations studied. We conducted this study to ascertain the prevalence of CD in patients with AT from Sardinia, an area with a very high prevalence of CD. To this aim, 297 consecutive patients with AT (as defined by elevated antithyroid antibody levels and a positive ultrasound scan) were studied. Immunoglobulin A and G-class antigliadin antibodies were assayed in serum; if either

An interesting case of pachydermoperiostosis with idiopathic myelofibrosis associated with monosomy 22.

A 24-year-old man was referred to our clinic in August 2003 with complaints of weakness, dizziness, and bilateral knee pain of 3 years' dura … ltrasonography revealed hepatosplenomegaly. Amyloid deposition was not determined in rectal biopsy. Reticulin-type myelofibrosis was found on bone marrow biopsy (Figs 4 and 5). In the cytogenetic study, monosomy 22 was detected in four of 20 metaphase plates.

[Clinicopathological features and prognosis of aggressive natural killer-cell leukemia: an analysis of 27 cases].

Objective: To investigate the clinicopathological features, diagnosis, and prognosis of aggressive natural killer-cell leukemia (ANKL). Meth … rom 50% to 90%. CD56-EBER dual staining showed that NK cells were the primary targets of the virus. Reticulin staining showed increased fibrosis. By flow cytometry, all cases were positive for CD2 but negative for surface CD3 (sCD3), CD4, CD5 and CD57. Among them, 21 cases (95.5%) exhibited a typical phenotype of strong CD56 expression (CD56str+) with CD16 …

Molecular docking and experimental evaluation of natural alkaloids from Chilean flora (Cryptocarya alba, Peumus boldus, and Laurelia sempervirens) for tyrosinase inhibition and depigmenting potential.

BACKGROUND: Hyperpigmentation disorders stem from tyrosinase-catalyzed melanin overproduction, worsened by ultraviolet oxidative stress. The … METHODS: Boldine (BOL), N-methyllaurotetanine (NMLT), laurolitsine (LTS), laurotetanine (LTT), and reticuline (RET) alkaloids were isolated from authenticated plant material via acid-base partitioning/silica gel chromatography; structures were confirmed by 1H-NMR/UHPLC-MS/MS (98% HPLC purity). Coclaurine (CC), N-methylcoclaurine (NMCC), and BOL derivatives …

In vitro and in vivo studies of the therapeutic potential of Tinospora crispa extracts in osteoarthritis: Targeting oxidation, inflammation, and chondroprotection.

ETHNOPHARMACOLOGICAL RELEVANCE: The increasing incidence of osteoarthritis (OA), especially among the elderly population, highlights the nee … S in positive ionization, identifying seven compounds, including pseudolaric acid B, stylopine, and reticuline, which were reported for the first time in this species. The study utilized varying concentrations of TC stem extracts, specifically 6.25-25 μg/mL for in vitro assays and 500 mg/kg for in vivo studies. Our findings also revealed that both ETCE and …

Clinical, histopathologic, and genetic features of pediatric primary myelofibrosis--an entity different from adults.

Primary myelofibrosis is a chronic myeloproliferative neoplasm characterized by cytopenias, leukoerythroblastosis, extramedullary hematopoiesis, hepatosplenomegaly and bone marrow fibrosis. Primary myelofibrosis is a rare disorder in adults; children are even less commonly affected by this entity, with the largest pediatric case series reporting on three patients. Most literature suggests spontaneous resolution of myelofibrosis without long term complications in the majority of affected children. We describe the clinical, pathologic, and molecular characteristics and outcomes of nineteen child

[Morphologic observation and pathogenesis investigation of regenerated sinusoidal endothelial cells in remodelling rat hepatic necrotic tissue].

OBJECTIVE: To investigate the morphological changes and regeneration mechanism of sinusoidal endothelial cell. METHODS: Sixty male Wistar r … ere transformed from round to spindle in shape. On the 7th day, these cells contacted with residual reticulin fibers and became positive for SE-1, a marker of hepatic sinusoidal endothelial cells and Tie-1, an endothelial cell-specific surface receptor, associated with frequent occurrence of ED-1/SE-1 and ED-1/Tie-1 double positive spindle cells. On the 8th …

Pentadecapeptide BPC 157 cream improves burn-wound healing and attenuates burn-gastric lesions in mice.

The effects of the gastric pentadecapeptide BPC 157 were investigated when administered topically or systemically in burned mice. This agent … ecrosis was seen with an increased number of capillaries along with an advanced formation of dermal reticulin and collagen fibers. An increased number of preserved follicles were observed. Two weeks after injury, BPC 157 cream-treated mice completely reversed the otherwise poor re-epithelization ratio noted in the untreated control or mice treated with vehi …

Prognostic utility of macrophage polarization (CD68/CD163 ratio) in Egyptian JAK2 positive myeloproliferative neoplasm patients: a single center study.

BackgroundMyeloproliferative neoplasms (MPNs) are clonal hematopoietic disorders with variable clinical outcomes influenced by the bone marrow microenvironment. Tumor-associated macrophages (TAMs), particularly the M1 (CD68⁺) and M2 (CD163⁺) subtypes, play critical roles in inflammation, fibrosis, and immune modulation. This study evaluates CD68- and CD163-positive macrophage frequencies across MPN subtypes and their clinical/prognostic significance.MethodsIn this retrospective cohort study, 121 patients with histopathologically confirmed BCR::ABL1-negative, JAK2V617F-positive MPNs were assess

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… The hematoxylin- and eosin-stained, silver-stained reticulin, and trichrome slides of the selected cases were reviewed for histopathologic data, including the histologic kind of neoplasm, reticulin and collagen fibrosis grade. … Hematoxylin and eosin stain, original magnification ×100 Fig. 2 Reticulin staining in primary myelofibrosis. (A, B) Coarse intersecting bundles of black-stained fibers. Reticulin silver stain, original magnification ×100 Fig. 3 Collagen staining in primary myelofibrosis. …

Romiplostim as a treatment for immune thrombocytopenia: a review.

"Immune thrombocytopenia" (ITP) is an autoimmune disorder that leads to peripheral destruction, as well as a decreased production of platelets. ITP most commonly presents as mild mucocutaneous bleeding. Though it is rare, the leading cause of mortality in persons with ITP is intracranial hemorrhage and those that do not respond to therapy are at increased risk. Our understanding of the pathophysiology of ITP has evolved immensely, especially over the last 60 years. The discovery of the platelet-production stimulator, thrombopoietin (TPO), lent clarity to an earlier hypothesis that inhibition o

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… Though romiplostim is generally well-tolerated, some potentially concerning side effects such as bone-marrow reticulin accumulation and thrombosis have been documented, justifying ongoing surveillance. … Bone marrow reticulin and collagen content in patients with adult chronic immune thrombocytopenic purpura: a Danish nationwide study. Am J Hematol. 2010;85(12):930–934. doi: 10.1002/ajh.21885. 69. Beckman EN, Brown AW., Jr Normal reticulin level in iliac bone marrow. …

Bone marrow fibrosis in newly diagnosed multiple myeloma and its correlation with clinicopathological factors.

BACKGROUND: Bone marrow fibrosis (BMF) severely impacts both the quality of life and the efficacy of diagnostic procedures. However, the correlation between BMF and clinicopathological features, cytogenetic changes, and prognosis of newly diagnosed multiple myeloma (NDMM) remains unclear. This study determined the incidence, patient characteristics, and clinical outcomes of patients with NDMM with BMF. METHODS: The clinical data, histological features, and clinical outcomes of patients with NDMM were collected. Reticular fiber staining was performed on the enrolled cases, and the degree of re

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… Reticulin is a normal component of the BM that may be increased in BM biopsy samples in a wide variety of neoplastic and non-neoplastic conditions. Bone marrow fibrosis (BMF) refers to the deposition of reticulin or collagen in the BM stromal environment beyond the normal range [ 9 ]. … The criteria were established as follows: MF-0, indicating scattered linear reticulin without intersections (cross-overs); MF-1, characterized by a loose network of reticulin with numerous intersections, predominantly in perivascular areas; MF-2, defined by a diffuse and dense increase in reticulin with …

Megakaryocyte Contribution to Bone Marrow Fibrosis: many Arrows in the Quiver.

In Primary Myelofibrosis (PMF), megakaryocyte dysplasia/hyperplasia determines the release of inflammatory cytokines that, in turn, stimulate stromal cells and induce bone marrow fibrosis. The pathogenic mechanism and the cells responsible for progression to bone marrow fibrosis in PMF are not completely understood. This review article aims to provide an overview of the crucial role of megakaryocytes in myelofibrosis by discussing the role and the altered secretion of megakaryocyte-derived soluble factors, enzymes and extracellular matrices that are known to induce bone marrow fibrosis.

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… Introduction Bone marrow (BM) fibrosis is characterized by increased deposition of reticulin fibres and in some cases collagen fibres. 1 There are a number of hematologic and non-hematologic disorders that are associated with increased BM fibrosis. … Evaluation of bone marrow reticulin formation in chronic immune thrombocytopenia patients treated with romiplostim. Blood. 2009;114(18):3748–3756. doi: 10.1182/blood-2009-05-224766. 74. Villeval JL, Cohen-Solal K, Tulliez M, et al. …

Синхронное тройное злоумышленничество с множественными миеломами и префиброзным первичным миелофиброзом у пациента, больного мухой молочной карциномой: доклад по делу из сельских районов Южной Африки.

Множественные первичные злокачественные новообразования (МПМН), диагностированные синхронно, встречаются редко и имеют частоту менее 1%. Происхождение тройных синхронных маньяков является чрезвычайно редким, и, насколько нам известно, ни в одном из предыдущих докладов не описывается одновременное представление о новообразовании миэлопролиферативной системы, дискразии плазменных клеток и рака молочной железы. Такие случаи сопряжены со значительными диагностическими и терапевтическими проблемами, особенно в условиях ограниченного доступа к передовым молекулярным тестам и онкологической терапии.

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… (C) Normal scattered linear reticulin, grade MF‐0 (reticulin stain × 10). (D) Absence of intertrabecular collagen deposition (Masson’s trichrome × 10). (E) Normal plasma cell number and distribution (CD138 IHC × 10). (F) Cytoplasm positive in plasma cells, with a normal proportion (kappa IHC × 10). … The marked megakaryocytic atypia, persistent leucocytosis and absence of reticulin fibrosis support a diagnosis of pre‐PMF over essential thrombocythaemia, in line with current WHO Haem 5 criteria and expert opinion [ 12 , 16 ]. …

A Phase Ib Investigator-Initiated Trial of Filgotinib in Patients With Idiopathic Multicentric Castleman Disease.

Background Idiopathic multicentric Castleman disease (iMCD) is a chronic systemic inflammatory disease characterized by the production of interleukin (IL)-6. The contribution of Janus kinase, downstream of IL-6 signaling, to the pathophysiology of iMCD has been suggested by several studies. Patients and methods This phase Ib single-arm trial evaluated the safety profile and efficacy of filgotinib, a JAK1 preferential inhibitor, in patients with iMCD. We recruited patients with disease activity evaluated based on their values of C-reactive protein (CRP), hemoglobin, albumin, and Eastern Coopera

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… Table 1 Exclusion criteria eGFR - estimated glomerular infiltration rate; iMCD - idiopathic multicentric Castleman disease; JAK - Janus kinase; TAFRO - the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype; TNF-α - tumor necrosis factor-alpha … Definite iMCD-TAFRO defined by "validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype of idiopathic multicentric Castleman disease" 11. …

Синдром опухоли с синдромом венетоклакса/Карфилзомиба/Дексаметазона для релапсированной/отредактированной миеломы: доклад по делу.

Фон и клиническая сенсибилизация: синдром опухоли (TLS) является редким явлением у пациентов, которые лечатся моно- или комбинированной терапией, и отсутствуют четкие протоколы, регулирующие профилактику TLS. Представление дела: Мы представляем 53-year-old мужского пола с историей рецидива рефрактора (RRMM) с t ()11;14) обработанные венетоклаксом, карфилзомидом и дексаметазоном (VenKd), что приводит к почечной недостаточности. Повторяющаяся биопсия мозга не показала ни моноклональных плазменных клеток, ни обширного фиброза. Венетоклакс был восстановлен после two месяцев с восстановлением мозга

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… fibrosis (Reticulin special stain, 100× magnification). ( D ) Focal coarse bundles of collagen fibers consistent with focal WHO grade MF-3 fibrosis in a background of grade MF-2 fibrosis (trichrome stain, 100× magnification). … Reticulin and trichrome stains demonstrated WHO grade 1 fibrosis, suggesting partial resolution of the marrow fibrosis. …

Thrombopoietin Receptor Agonists: Can These Be the Future Answer to the Deadly Thrombocytopenia in Dengue Fever?

Dengue is considered the most prevalent mosquito-borne viral disease worldwide and sometimes turns out to be life-threatening. Thrombocytopenia is frequently observed in mild and severe cases of dengue. Severe thrombocytopenia, with a platelet count much below the normal range and hemorrhagic manifestation, is considered a fatal consequence of dengue that needs proper and timely management. The development of the dengue vaccine is quite challenging due to the existence of four different serotypes of the virus. Currently, neither a specific antiviral therapy nor a vaccine is available, and the

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… Only two romiplostim-treated patients reported adverse reactions involving increased bone marrow reticulin in one patient and peripheral disease and atrial fibrillation in another patient. However, bone marrow reticulin returned to the baseline level on the discontinuation of romiplostim [ 35 ]. … Few patients showed increased bone marrow reticulin; only three patients developed neutralizing romiplostim antibodies but no endogenous TPO antibody [ 38 ]. …

Orbital solitary fibrous tumor: a clinicopathologic study of ten cases with long-term follow-up.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare spindle-cell benign neoplasm and located in orbit. The present research represents case reports of ten patients with orbital SFT. METHOD: Prognosis was assessed by clinic service and telephone interview. Clinical data were retrieved from the medical records. Immunohistochemistry for CD34, CD99, EMA, HMB-45, Bcl-2, GFAP, S-100, MBP, CK, and MIB-1 was performed. Distributions of time to progression and recurrence between different operative methods were estimated using the Kaplan-Meier method and compared using the log-rank test. FINDINGS: The

Dissecting Bone Marrow Fibrosis in Philadelphia-Negative Myeloproliferative Neoplasms: Multiple Roads Lead to Rome.

Philadelphia-negative myeloproliferative neoplasms (MPN), a spectrum of blood malignancies characterized by uncontrolled myeloid proliferation, persist as a key challenge in onco-hematology due to their intricate molecular background, heterogeneous clinical phenotypes, and variable prognoses. Although generally accepted as indolent disorders, a considerable fraction of MPN patients exhibit progressive, and sometimes accelerated, bone marrow fibrosis, reflecting a critical shift toward a more debilitating condition marked by an increased symptom burden. As marrow scarring advances, hematopoieti

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… FIGURE 2 Representative bone marrow sections from a case of prefibrotic myelofibrosis showing clustered atypical megakaryocytes and perimegakaryocytic reticulin reinforcement. … (B) The corresponding reticulin stain (× 25) demonstrates conspicuous reinforcement of the reticulin framework surrounding both clusters and individual megakaryocytes, outlining early perimegakaryocytic stromal remodeling typical of megakaryocytic activation. …