Concurrent myelodysplastic malignancies and plasma cell neoplasms; a clinicopathological study with prognostic implications.

Plasma cell neoplasms (PCN) have infrequently been reported in patients with myelodysplastic syndrome (MDS) and even more rarely in those with myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN). We report the clinicopathologic features of 26 patients with bone marrow myelodysplasia accompanied by PCN, including 21 patients with MDS and 5 with MDS/MPN. The clinicopathologic features of the MDS/MPN-PCN were compared to those of the MDS-PCN group and 68 cases of MDS/MPN without PCN, respectively.

Advances and Progress in Automated Urine Analyzers.

The clinical analysis of urine has classically focused on conventional chemical-based urinalysis and urine microscopy. Contemporary advances in both analysis subsets have started to employ new technologies such as automated image analysis, flow cytometry, and mass spectrometry. In addition to new detection technologies, current analyzers have incorporated more advanced imaging, automated sample handing, and machine learning analyses into their workflow.

Assessment for acceleration and transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma using histologic and immunohistochemical features: a case series.

Morphologic features of aggressive/ "accelerated" chronic lymphocytic leukemia/small lymphocytic lymphoma (aCLL/SLL) have been described. Richter transformation (RT) also occurs in a subset of CLL/SLL cases. This case series examined inter-observer variability when assessing for aCLL/SLL and RT, with attention to how immunohistochemical (IHC) markers may assist in this evaluation.

Germline Predisposition to Myeloid Neoplasms: Diagnostic Concepts and Classifications.

Molecular and sequencing advances have led to substantial breakthroughs in the discovery of new genes and inherited mutations associated with increased risk of developing myeloid malignancies. Many of the same germline mutated genes are also drivers of malignancy in sporadic cancer. Recognition of myeloid malignancy associated with germline mutations is essential for proper therapy, disease surveillance, informing related donor selection for hematopoietic stem cell transplantation, and genetic counseling of the patient and affected family members.

Aggressive non-Hodgkin lymphoma in the pediatric and young adult population; diagnostic and molecular pearls of wisdom.

Mature non-Hodgkin lymphomas (NHLs) of the pediatric and young adults(PYA), including Burkitt lymphoma (BL), diffuse large B cell lymphoma (DLBCL), high-grade B cell lymphoma (HGBCL), primary mediastinal large B cell lymphoma (PMBL) and anaplastic large cell lymphoma (ALCL), generally have excellent prognosis compared to the adult population. BL, DLBCL and HGBCL are usually of germinal center (GCB) origin in the PYA population. PMBL neither belongs to the GCB nor the activated B cell subtype and is associated with a poorer outcome than BL or DLBCL of comparable stage.

Diagnostic Flow Cytometry in the Era of Targeted Therapies: Lessons from Therapeutic Monoclonal Antibodies and Chimeric Antigen Receptor T-cell Adoptive Immunotherapy.

Therapeutic monoclonal antibodies (therapeutic mAb) and adoptive immunotherapy have become increasingly more common in the treatment of hematolymphoid neoplasms, with practical implications for diagnostic flow cytometry. Their use can reduce the sensitivity of flow cytometry for populations of interest owing to downregulation/loss of the target antigen, competition for the target antigen, or lineage switch. Expanded flow panels, marker redundancy, and exhaustive gating strategies can overcome this limitation.

Isolated Light Chain-restricted Germinal Centers are Common in Follicular Hyperplasia by Ultrasensitive In Situ Hybridization.

Ultrasensitive bright-field RNA in situ hybridization (BRISH) can be used to detect lower levels of light chain expression than immunohistochemical stains or conventional colorimetric RNA in situ hybridization. In this study, we retrospectively reviewed 77 lymph node specimens with follicular hyperplasia and kappa/lambda BRISH performed as part of the diagnostic evaluation. Thirty-two of the specimens had ≥1 germinal center(s) (GC) showing light chain restriction (14 specimens with lambda-restricted GC, 9 with kappa-restricted GC, and 9 with separate kappa-restricted or lambda-restricted GC).

Overlapping Features of Primary Cutaneous Marginal Zone Lymphoproliferative Disorder and Primary Cutaneous CD4 + Small/Medium T-Cell Lymphoproliferative Disorder : A Diagnostic Challenge Examined by Genomic Analysis.

Primary cutaneous marginal zone lymphoproliferative disorder (PCMZL) and primary cutaneous CD4 + small/medium T-cell lymphoproliferative disorder (CD4 + TLPD) are indolent lymphoproliferative disorders. However, cases with overlapping features can be challenging. We identified 56 CD4 + TLPD and 38 PCMZL cases from our pathology archives.

The Effectiveness of Dual-Staining Immunohistochemistry in the Detection of Mantle Cell Lymphoma in the Bone Marrow.

Objectives: Immunohistochemistry (IHC) is a useful method for mantle cell lymphoma (MCL) detection in the bone marrow (BM). However, recognition of the neoplastic B cells can be challenging, especially when there is low-level disease.

Methods: We examined BM from 105 patients with MCL.

Benign and malignant hematologic manifestations in patients with VEXAS syndrome due to somatic mutations in UBA1.

Somatic mutations in UBA1 involving hematopoietic stem and myeloid cells have been reported in patients with the newly defined VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome. Here, we report clinical hematologic manifestations and unique bone marrow (BM) features in 16 patients with VEXAS. All patients were male and had a history of severe autoinflammatory and rheumatologic manifestations and a somatic UBA1 mutation (p.

Concurrent chronic lymphocytic leukemia/small lymphocytic lymphoma and hairy cell leukemia: clinical, pathologic and molecular features.

Simultaneous occurrence of hairy cell leukemia (HCL) and chronic lymphocytic leukemia/small lymphocytic lymphoma (termed CLL) is very rare. Clinical characteristics, pathology and management of these cases have not been well described. We present six patients with CLL and HCL or HCL variant (HCL-v).

Well differentiated arginase-1 negative hepatocellular carcinoma.

Background: The diagnosis of hepatocellular carcinoma (HCC) is dependent on the histologic and immunohistochemical analysis of biopsy and resection specimens. The distinction of HCC from metastatic neoplasms is pertinent for treatment and prognostic purposes. Arginase-1 (Arg-1), a marker of hepatocellular differentiation, has shown superior sensitivity and specificity when compared to other immunohistochemical markers of detection of HCC such as hepatocyte paraffin antigen (HepPar-1).

Clear cell sarcoma in unusual sites mimicking metastatic melanoma.

Background: Clear cell sarcoma is an aggressive rare malignant neoplasm with morphologic and immunohistochemical similarities to malignant melanoma. Both disease entities display melanin pigment and melanocytic markers, making differentiation between the two difficult. Although clear cell sarcoma cases in the literature have mainly involved deep soft tissues of the extremities, trunk or limb girdles, we report here two cases of primary clear cell sarcoma in unusual sites and describe their clinicopathologic findings.

Prognostic Implications of Arginase and Cytokeratin 19 Expression in Hepatocellular Carcinoma After Curative Hepatectomy: Correlation With Recurrence-Free Survival.

Background: The prognostic value of arginase expression in hepatocellular carcinoma (HCC) has been evaluated previously. However, no clear distinction exists yet on the role of arginase-1 as a predictor of recurrence in HCC. Cytokeratin 19 (CK19), a cholangiocytic marker, is occasionally expressed in HCC, but the combination of arginase-1 and CK19 expression has never been evaluated.

Radiation-induced astrocyte senescence is rescued by Δ133p53.

Background: Cellular senescence and the senescence-associated secretory phenotype (SASP) may contribute to the development of radiation therapy-associated side effects in the lung and blood vessels by promoting chronic inflammation. In the brain, inflammation contributes to the development of neurologic disease, including Alzheimer's disease. In this study, we investigated the roles of cellular senescence and Δ133p53, an inhibitory isoform of p53, in radiation-induced brain injury.

The Clinicopathologic Spectrum of IgG4-Related Disease.

Immunoglobulin G4-related disease is a fibroinflammatory systemic disease that is characterized by focal or diffuse organ infiltration by immunoglobulin G4-bearing plasma cells. Immunoglobulin G4-related disease may affect any organ, and a high index of suspicion is necessary for early detection to avoid irreversible fibrosis, organ dysfunction, and death. Tumor-forming lesions are common radiological features of immunoglobulin G4-related disease that need to be differentiated from malignancies.

Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis.

Intravascular lymphoma is a rare type of lymphoma, characterized by growth of lymphoma cells within the microvasculature. The majority of the cases are of B-cell lineage, although rare examples of T or NK lineage have also been reported. The lymphoma is usually widely disseminated in the vascular spaces of any organ at the time of diagnosis including the skin and bone marrow.

Incidental Metastatic Meningioma Presenting as a Large Liver Mass.

Meningiomas are slow growing neoplasms of the central nervous system (CNS). Most of the tumors are benign and distant metastasis from a benign meningioma is rare. Metastasis to the liver, although rare, usually presents with hypoglycemia or occurs in conjunction with a clinical history of an intracranial meningioma or following the resection of a prior CNS meningioma, thus making clinical diagnosis relatively easy.

Myeloid sarcoma with megakaryoblastic differentiation presenting as a breast mass.

Myeloid sarcoma is an extramedullary tumor that consists of myeloblasts or immature myeloid cells. The neoplasm can occur in any part of the body, including the bone, periosteum, lymph nodes, skin, and soft tissue and they may occur de novo or in association with acute myeloid leukemia, myeloproliferative neoplasms and myelodysplastic syndromes. Most cases display a myelomonocytic or pure monoblastic morphology.

Synchronous Leydig Cell Tumor and Seminoma in the Ipsilateral Testis.

Leydig cell tumor is a rare sex cord tumor that accounts for 1-3% of all testicular neoplasms. Seminomas are more common and occur in 30-40% of testicular tumors. Leydig cell tumors are derived from undifferentiated gonadal mesenchyme and the concurrent development of the tumor and a seminoma which are derived from germinal epithelium in an ipsilateral testis is extremely rare.

Primary Langerhans Cell Histiocytosis of the Extrahepatic Bile Duct Occurring in an Adult Patient.

Background: Langerhans cell histiocytosis is characterized by an abnormal proliferation of neoplastic Langerhans cells. Langerhans cell histiocytosis commonly affects the pediatric population, whereas presentation in adults remains a rare event. The presentation of Langerhans cell histiocytosis is highly variable, but the involvement of skin, bone, and lung is very common.