Duodenal Ulcer with Massive Gastrointestinal Hemorrhage as an Initial Manifestation in Multiple Myeloma with Extramedullary Disease: A Case Report

Plasma cell neoplasms are characterized by dysregulated proliferation of mature B cells, which can present with either single (solitary plasmacytoma) or systemic (multiple myeloma (MM)) involvement. MM with extramedullary plasmacytoma (EMP) is a rare disease that accounts for approximately 3–5% of all plasmacytomas. EMP with gastrointestinal (GI) system involvement is an even rarer entity, accounting for <1% of MM cases. We present a case of aggressive MM with EMP invading the duodenum, initially presented with massive upper GI hemorrhage and small bowel obstruction. A 67-year-old woman was admitted to our hospital owing to a lack of either gas or feces passage for 3 days. Abdominal distention and vomit with a high coffee ground content were observed for 24 h. The patient’s condition was initially diagnosed as small bowel obstruction, upper gastrointestinal bleeding, severe anemia, acute renal failure, and hypercalcemia. Furthermore, an analysis of immunoelectrophoresis in the blood, bone marrow aspiration, and tissue biopsy supported the diagnosis of MM and EMP invading the duodenum, upper GI hemorrhage, and small bowel obstruction. Our study provided the possible involvement of MM and EMP in the differential diagnosis of patients with unexplained GI hemorrhage and small bowel obstruction. A thorough review of the literature regarding the association between MM, GI hemorrhage, and small bowel obstruction is presented in this study.

BRAF V600E Positive Hairy Plasma Cell Leukemia; Are the Cytoplasmic Projections in Plasma Cells Predictive of a Particular Molecular Characterization?

Introduction: Plasma cell leukemia (PCL) is a rare and clinically aggressive form of plasma cell dyscrasia. Despite the significant role of BRAF mutation in plasma cell neoplasms, this mutation has been rarely considered in these cases. Finding evidence guiding us toward assessing the BRAF mutation in patients with plasma cell neoplasms could help make the suitable decision for targeted therapy. Case Presentation: A 79-year-old man presented with leukocytosis. Peripheral blood smear exhibited marked lymphocytosis and infiltration of about 50% abnormal lymphoid cells with slender cell-surface projections and oval shape nucleus. These findings raised the provisional diagnosis of hairy cell leukemia (HCL) or HCL variants (HCL-v). Molecular analysis confirmed the presence of BRAFV600E mutation, which was in agreement with HCL diagnosis, albeit the flow cytometric assessment of abnormal lymphocytes corroborated PCL. Conclusions: Together with the previous comprehensive analysis regarding the association of cytoplasmic projections and BRAF mutations, our findings could suggest this morphological characteristic in plasma cells (PCs) as an indication for the assessment of BRAF V600E mutation in PC dyscrasias.

Tumor Microenvironment of Lymphomas and Plasma Cell Neoplasms: Broad Overview and Impact on Evaluation for Immune Based Therapies

Lymphomas and plasma cell neoplasms are a heterogenous group of malignancies derived from lymphocytes. They are a significant cause of patient morbidity and mortality. Advances in morphologic, immunophenotypic and molecular techniques have led to better understanding of the pathogenesis and diagnosis of these neoplasms. Advances in treatment, particularly immune-based therapies, increasingly allow for targeted therapies of these diseases. Mechanistic studies using animal models and clinical trials have revealed the importance of the tumor microenvironment on disease pathogenesis, progression, and response to therapy in these malignancies. Simultaneous progress in diagnostic techniques has made it feasible to generate high-resolution, high-throughput data from the tumor microenvironment with spatial context. As the armamentarium of targeted therapies and diagnostic techniques grows, there is potential to harness these advances to better stratify patients for targeted therapies, including immune-based therapies, in hematologic malignancies.

Mitochondria and Their Relationship with Common Genetic Abnormalities in Hematologic Malignancies

Hematologic malignancies are known to be associated with numerous cytogenetic and molecular genetic changes. In addition to morphology, immunophenotype, cytochemistry and clinical characteristics, these genetic alterations are typically required to diagnose myeloid, lymphoid, and plasma cell neoplasms. According to the current World Health Organization (WHO) Classification of Tumors of Hematopoietic and Lymphoid Tissues, numerous genetic changes are highlighted, often defining a distinct subtype of a disease, or providing prognostic information. This review highlights how these molecular changes can alter mitochondrial bioenergetics, cell death pathways, mitochondrial dynamics and potentially be related to mitochondrial genetic changes. A better understanding of these processes emphasizes potential novel therapies.

A case of solitary plasmacytoma of bone showing co-expression of both immunoglobulin light chains

Background Solitary plasmacytoma of bone (SPB) is a rare plasma cell neoplasm. It arises in bone as a single locus in the absence of any plasma cell myeloma lesions. Plasma cell neoplasms intrinsically express only one immunoglobulin light chain (IgL)—kappa or lambda—and using this fact, kappa/lambda deviation is the decisive factor for diagnosis. Co-expression of both IgLs in a single tumor cell is extremely rare. Case presentation We report a case of SPB that arose in the vertebra of a 52-year-old Japanese woman. Histologically, the resected mass showed diffuse plasma cell proliferation. Dual IgL expression was detected by flow cytometry, immunohistochemistry, and in situ hybridization (ISH) targeting IgL mRNA. Conclusion We have presented an extremely rare case of SPB showing dual expression of kappa and lambda IgLs. This unusual case of plasma cell neoplasia might represent a possible exceptional example of failure of “IgL isotypic exclusion.”

Exploring Solitary Bone Plasmacytoma: A Rare Case Report

The Solitary Bone Plasmacytoma (SBP) is a rare oncologic disease corresponding to less than 5% of the malignant neoplasms of the plasma cells. It is characterized as a localized aggressive tumor consequent to the accumulation of monoclonal plasma cell neoplasms and, due to the rarity of the disease, there are only few clinical studies reporting it especially regarding prognostic factors and treatment. Here, we report a fatal clinical case of SPB in Roraima, the northernmost state of Brazil. The study shows a case of solitary bone plasmacytoma on the femur, where the patient was submitted to the preconized radiotherapy cycles. However, over approximately four-years following the diagnose, the patient required multi-modal approach to guarantee quality of life during the survival time. Finally, this study explores SBP-related issues and examines the challenges physicians face when managing the care of patients with SBP.

Extramedullary plasmacytoma of the tongue: A case report

Background: Tongue extramedullary plasmacytoma is a very rare pathology. Despite rare cases, extramedullary plasmacytoma should be considered as a differential diagnosis in case of a mass in the tongue. A total of 19 cases were reported with EMP in English literature along with the case we will address. It is characterized by a monoclonal neoplastic proliferation of plasma cells in the absence of multiple myeloma (MM). Histopathology and immunohistochemistry are very important for the diagnosis and differential diagnosis. Case presentation: The case we will describe is an 80-year-old lady from Albania who presents with a vegetative lesion in the form of a thick plate on the dorsal part of the tongue with dimensions 6 X 5 X 1.5 cm. A material of 0.5 cm diameter was taken from the lesion for the biopsy. After histopathological examination, immunohistochemical examinations, and after correlations with laboratory, clinical and imaging data, the diagnosis of extramedullary plasmacytoma of the tongue was reached. The patient underwent radiotherapy treatment. Conclusions: EMP is a rare tumor, accounting for 3% of plasma cell neoplasms and <1% of all head and neck tumors. The diagnosis of EMP, in this case, was reached with biopsy, immunohistochemistry, and the correlation with laboratory and imaging data. We will show the importance of biopsy along with immunohistochemistry in the diagnosis and differential diagnosis of extramedullary plasmocytoma of the tongue. Keywords: plasmacytoma; immunohistochemistry; biopsy; plasma cell. Abbreviations: EMP: Extramedullary plasmacytoma; MM: Multiple myeloma; Cm: centimeter

Plasmacytoma of the mandible: A diagnostic conundrum

Plasma cell dyscrasias are neoplastic proliferation of monoclonal plasma cells that encompass a wide range of entities. Plasmacytoma may present as one of two distinct clinical entities: Multiple myeloma and solitary plasmacytoma. The incidence of solitary plasmacytoma is 2-5% among all plasma cell neoplasms and it commonly involves long bones and vertebrae. Its occurrence in the jaw is extremely rare and only 4.4% are seen in mandible. Clinically, plasmacytoma of mandible presents as pain, tooth migration, hard and soft tissue swelling or pathological fracture. Radiologically, it presents as unilocular or multilocular lesion mimicking odontogenic tumour creating a diagnostic dilemma.We report a series of 3 cases of plasmacytoma of the mandible with comprehensive details of clinico-radiological, histological, immunohistochemistry findings and treatment with a review of the literature.These three cases were clinically mistaken for Ameloblastoma, Odontogenic tumour and Oral cancer respectively. There was one case of Solitary Plasmacytoma and two cases on further workup proved to be multiple myeloma. All these cases were confirmed by immunohistochemistry.Plasmacytoma of mandible is very rare. It is usually mistaken for other common mandibular lesions and oral cancers. Every attempt must be made to diagnose them precisely as management is quite specific. To best of our knowledge, this is the first series of plasmacytoma of the mandible in the Indian literature.