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Acute Pancreatitis as a Rare Initial Presentation of Plasma Cell Leukemia in a Young Male: A Case Report.

Plasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm characterized by the presence of neoplastic plasma cells in the peripheral blood. It may arise de novo (primary PCL) or may evolve from a previously existing plasma cell neoplasm (secondary PCL). It is typically a disease of old age, with a median age of incidence of approximately 55 years. Primary PCL is often considered a manifestation of extra-medullary myeloma (EMM) and is typically associated with extensive organ involvement, while lacking extensive osteolytic lesions. We present the case of a 24-year-old male patien

Severity of Chronic Graft-versus-Host Disease and Late Effects Following Allogeneic Hematopoietic Cell Transplantation for Adults with Hematologic Malignancy.

The study aimed to determine the association of chronic graft-versus-host disease (cGVHD) diagnosis and severity with the development of subsequent neoplasms (SN) and nonmalignant late effects (NM-LE) in 2-year disease-free adult survivors following hematopoietic cell transplantation (HCT) for a hematologic malignancy. To do so, we conducted a retrospective analysis of 3884 survivors of HCT for hematologic malignancy in the Center of International Blood and Marrow Transplant Research database. We conducted a landmark analysis at the 2-year post-transplantation date, comparing first SN and NM-L

Safety and efficacy of combination therapy of interferon-α2 and ruxolitinib in polycythemia vera and myelofibrosis.

Interferon-α2 reduces elevated blood cell counts and splenomegaly in patients with myeloproliferative neoplasms (MPN) and may restore polyclonal hematopoiesis. Its use is limited by inflammation-mediated toxicity, leading to treatment discontinuation in 10-30% of patients. Ruxolitinib, a potent anti-inflammatory agent, has demonstrated benefit in myelofibrosis (MF) and polycythemia vera (PV) patients. Combination therapy (CT) with these two agents may be more efficacious than monotherapy with either, potentially improving tolerability of interferon-α2 as well. We report the preliminary results

Cancer Therapy-associated Lymphoproliferative Disorders: An Under-recognized Type of Immunodeficiency-associated Lymphoproliferative Disorder.

We describe the clinicopathologic features of 17 patients who had a hematologic malignancy of various types, were treated, and subsequently developed a lymphoproliferative disorder (LPD). There were 10 men and 7 women with a median age of 59 years (range, 36 to 83 y). The primary hematologic neoplasms included: 5 chronic lymphocytic leukemia/small lymphocytic lymphoma, 3 plasma cell myeloma, 2 acute monoblastic leukemia, and 1 case each of mixed-phenotype acute leukemia, chronic myeloid leukemia, splenic marginal zone lymphoma, follicular lymphoma, mantle cell lymphoma, T-cell prolymphocytic l…

Asparaginase in the treatment of non-ALL hematologic malignancies.

Asparaginases are among the most effective agents against acute lymphoblastic leukemia (ALL) and are Food and Drug Administration-approved f … proliferation of individual malignant cells may identify a special group of patients whose solid or hematologic neoplasms may benefit significantly from interruption of glutamine metabolism. To this end, asparaginase products deserve a second look particularly in non-ALL malignant blood disorders. Here, we review mechanisms of anti-tumor activity of asparaginase focusi …

MPRIP::PDGFRB Fusion Gene: A Rare Case Report of Adult Myeloid/Lymphoid Neoplasm With Eosinophilia and Tyrosine Kinase Gene Fusions.

Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions (MLN-TK) represent rare hematological malignancies driven by pathological fusion genes involving tyrosine kinase genes. Among these, rearrangements of the PDGFRB gene, particularly the ETV6::PDGFRB rearrangement, are frequently observed as pathogenic mutations. Conversely, instances of the MPRIP::PDGFRB fusion gene are rarely documented. In this case report, we present a 32-year-old previously healthy Thai male who presented to the hospital with constitutional symptoms and marked splenomegaly. His complete blood coun

Mucocutaneous Findings in Hematolymphoid Neoplasms: An Observational Study.

BackgroundCutaneous manifestations of hematological neoplasms can be divided into three broad categories - direct infiltration, paraneoplastic conditions, and those due to the treatment of hematological cancers.ObjectivesTo study the frequency and patterns of mucocutaneous manifestations in patients with hematolymphoid neoplasms and those due to chemotherapy.Materials and methodsThis was an observational study done with 172 patients. Categorization of mucocutaneous manifestations was done into malignancy-associated and chemotherapeutic drugs-associated and data was analyzed.ResultsOut of a tot

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… Introduction Cutaneous manifestations of hematological neoplasms can be divided into three broad categories:[ 1 ] (a) Direct infiltration of skin with malignant cells or their products (e.g., paraproteins) (b) Paraneoplastic conditions or dermatological syndromes associated with underlying malignancy … Cutaneous manifestations and management of hematologic neoplasms. Semin Oncol. 2016;43:370–83. doi: 10.1053/j.seminoncol.2016.02.021. 9. Smith JL, Jr, Butler JJ. Skin involvement in Hodgkin's disease. …

Convalescent plasma improves overall survival in patients with B-cell lymphoid malignancy and COVID-19: a longitudinal cohort and propensity score analysis.

Patients with hematological malignancy and COVID-19 display a high mortality rate. In such patients, immunosuppression due to underlying disease and previous specific treatments impair humoral response, limiting viral clearance. Thus, COVID-19 convalescent plasma (CCP) therapy appears as a promising approach through the transfer of neutralizing antibodies specific to SARS-CoV-2. We report the effect of CCP in a cohort of 112 patients with hematological malignancy and COVID-19 and a propensity score analysis on subgroups of patients with B-cell lymphoid disease treated (n = 81) or not (n = 120)

Synchronous duodenal neuroendocrine neoplasm and congenital factor XIII deficiency: case report and review of the literature.

BACKGROUND: Neuroendocrine neoplasms (NENs) are uncommon, with duodenal NENs (dNENs) being particularly rare in clinical practice. Congenital factor XIII deficiency (FXIIID) is also an extremely rare hematological disease in which poor wound healing may occur due to coagulopathy. The concurrent occurrence of these two rare diseases has not been reported before, which increases the difficulty of diagnosis and treatment. This is the first report of dNEN concomitant with Congenital FXIIID, which can present as a reference for clinicians who may encounter similar situations in the future. CASE DE

Trichoblastomas derived from the facial skin with tactile hair in aged house musk shrews (Suncus murinus).

BACKGROUND: Benign hair follicle tumors are relatively rare cutaneous neoplasms arising from hair follicle differentiation. These tumors are slow-growing solitary papules or nodules in the head, face or neck. The aim of this study was to describe 2 cases of trichoblastomas in tactile hair skin incidentally encountered in aged house musk shrews (Suncus murinus). In addition, this case report clarifies whether the characteristics in the tactile hair skin of Suncus murinus are different from those in humans and other animals. CASE PRESENTATION: The animals were investigated the characteristics o

Checkpoint inhibitors in hematological malignancies.

Inhibitory molecules such as PD-1, CTLA-4, LAG-3, or TIM-3 play a role to keep a balance in immune function. However, many cancers exploit such molecules to escape immune surveillance. Accumulating data support that their functions are dysregulated in lymphoid neoplasms, including plasma cell myeloma, myelodysplastic syndrome, and acute myeloid leukemia. In lymphoid neoplasms, aberrations in 9p24.1 (PD-L1, PD-L2, and JAK2 locus), latent Epstein-Barr virus infection, PD-L1 3'-untranslated region disruption, and constitutive JAK-STAT pathway are known mechanisms to induce PD-L1 expression in lym

Budd-Chiari syndrome: reassessment of a step-wise treatment strategy.

BACKGROUND AND STUDY AIMS: The Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction. A step-wise management was recently proposed. The aim of this study is to reassess our treatment approach and long-term outcome. PATIENTS AND METHODS: The data of 37 Budd-Chiari patients, seen in our unit, were critically analyzed and compared with the ENVIE (European Network For Vascular Disorders of the Liver) data. RESULTS: Most patients had multiple prothrombotic conditions (41%), of which an underlying myeloproliferative neoplasm was the most frequent (59%). The JA

Malignancies occurring during therapy with tyrosine kinase inhibitors (TKIs) for chronic myeloid leukemia (CML) and other hematologic malignancies.

Success of tyrosine kinase inhibitors (TKIs) in chronic myeloid leukemia (CML) has given patients hope for a long disease-free-survival. A longer survival raises the question of late effects, including development of another malignancy. Records of 1445 patients with CML/myeloproliferative neoplasm or other hematologic malignancies treated with TKIs were reviewed to investigate frequency and characteristics of second malignancies (other than acute myeloid leukemia, acute lymphocytic leukemia, or myelodysplastic syndrome). The number of second cancers was compared with the number expected from t

Cutaneous CD4+ CD56+ hematologic malignancies.

BACKGROUND: Hematologic malignancies expressing CD4 and CD56 are most commonly associated with the recently described CD4(+) CD56(+) hematodermic neoplasm. METHODS: Thirteen cases of CD4(+) CD56(+) hematologic malignancies were prospectively encountered in the routine and referral practices of the authors. RESULTS: Patients 1 and 2 were elderly men exhibiting an acute onset of skin, bone-marrow, and peripheral blood involvement, both dying of their disease within less than 12 months. CD3(+) phenotype and a clonal T-cell receptor beta rearrangement indicated categorization as a CD4(+) natural

Family cancer history and risk of childhood acute leukemia (France).

OBJECTIVE: A case-control study was carried out to investigate the role of a family history of solid tumor or hematologic neoplasm in the etiology of childhood acute leukemia. METHODS: Family cancer history in first- and second-degree relatives was compared in 279 incident cases (242 cases of acute lymphocytic leukemia and 37 of acute myeloid leukemia) and 285 controls. Recruitment was stratified by age, gender, hospital, area of residence, and ethnic origin. Odds ratios (OR) were estimated using an unconditional regression model taking into account the stratification variables, socioeconomic

Molecular characterization of 12p abnormalities in hematologic malignancies: deletion of KIP1, rearrangement of TEL, and amplification of CCND2.

Twenty patients with hematologic malignancies with 12p abnormalities were investigated by fluorescence in situ hybridization (FISH) using probes mapped to specific regions in 12p. The initial analysis using the YAC 964c10 (D12S736) revealed that all four cases with cytogenetically identified del(12p) had lost one copy of this YAC and that submicroscopic deletions had occurred in 10 of the 16 neoplasms with other 12p abnormalities, ie, translocations, additions, and insertions. The deletions were partially mapped with cosmids localized to subregions of 12p. One copy of the gene for p27kip1 (KIP

Prevalence of chromosome 8p11.2 translocations and correlation with myeloid and lymphoid neoplasms associated with FGFR1 abnormalities in a consecutive cohort from nine institutions in Japan.

Myeloid and lymphoid neoplasms associated with FGFR1 abnormalities (MLN-FGFR1 abnormalities) are rare hematologic malignancies associated with chromosome 8p11.2 abnormalities. Translocations of 8p11.2 were detected in 10 of 17,039 (0.06%) unique patient cytogenetic studies performed at nine institutions in Japan. No inversions or insertions of 8p11.2 were detected. Among the 10 patients with 8p11.2 translocations, three patients were diagnosed with MLN-FGFR1 abnormalities, which were confirmed by FISH analysis. Peripheral blood eosinophilia was observed in all three patients, and all progresse

Hypercalcemia and urologic malignancies.

Hypercalcemia may be a manifestation of a variety of disorders including hyperparathyroidism, hypervitaminosis D, sarcoidosis, multiple myeloma, hyperthyroidism, acute osteoporosis, metastatic bone disease, and a number of primary malignancies. Hypercalcemia may be seen in as many as 1.5% of all patients with malignant disease, with or without bony metastases. The neoplasms most commonly associated with hypercalcemia include carcinoma of the lung (all cell types), breast cancer, squamous cell carcinomas, hematologic malignancies, and renal cell carcinoma. Observation of a number of instances o

Blastic plasmacytoid dendritic cell neoplasm.

Blastic plasmacytoid dendritic cell neoplasm is an uncommon, aggressive hematologic neoplasm carrying a poor prognosis with a median survival of one year, making early detection vital. Patients present with a number of characteristic cutaneous manifestations and are treated with chemotherapy and hematopoietic stem cell transplantation, which may improve survival. In this case, a 65-year-old man with a history of basal cell carcinoma presented with a nodule on his forehead with a honey-crusted border. Although the patient was treated with intralesional triamcinolone and a 7-day course of cephal

NPM1 -Mutated Myeloid Sarcoma/Leukemia Cutis Mimicking Blastic Plasmacytoid Dendritic Cell Neoplasm and Heralding Bone Marrow Acute Myeloid Leukemia.

Myeloid sarcoma and blastic plasmacytoid dendritic cell neoplasm (BPDCN) are hematologic malignancies of immature cells that frequently involve the skin. In the absence of bone marrow involvement or an associated myeloid neoplasm, distinguishing myeloid sarcoma with disseminated cutaneous involvement (leukemia cutis) from BPDCN is particularly challenging because of overlapping clinical and immunophenotypic features. We report a case of NPM1-mutated myeloid sarcoma presenting with rapidly progressive cutaneous lesions and no initial medullary disease, resulting in an initial misdiagnosis of BP