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Diagnostic criteria and classification of mastocytosis: a consensus proposal.

The term 'mastocytosis' denotes a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MC) in one or more organ systems. Over the last 20 years, there has been an evolution in accepted classification systems for this disease. In light of such developments and novel useful markers, it seems appropriate now to re-evaluate and update the classification of mastocytosis. Here, we propose criteria to delineate categories of mastocytosis together with an updated consensus classification system. In this proposal, the diagnosis cutaneous mastocytosis (CM) is

A classification based on T cell selection-related phenotypes identifies a subgroup of childhood T-ALL with favorable outcome in the COALL studies.

During T cell selection in the thymic cortex more than 90% of the thymocytes are eliminated by apoptosis. Based on this biology, we propose to define blasts of T cell acute lymphoblastic leukemia (ALL) with the phenotype of cortical thymocytes (CD1+ and/or CD4+ 8+) as selection-related (SR) and all other T-ALL immunophenotypes as non-selection-related (NSR). The COALL cooperative treatment studies for childhood ALL offer a tool to study the outcome in T-ALL subgroups as children with T-ALL are allocated uniformly to the high risk arm of the protocol. In the COALL-85, -89 and -92 protocols, 39/

CD56 expression predicts occurrence of CNS disease in acute lymphoblastic leukemia.

We examined the pre-treatment bone marrow samples from 200 consecutive adult patients with acute lymphoblastic leukemia (ALL) treated on various protocols at the University of Texas, M.D. Anderson Cancer Center between 1986 and 1998. Standard MFC techniques were used to determine CD56 expression on the leukemia blasts cells. The expression of CD56 was correlated with clinical characteristics at diagnosis, response to therapy, survival and disease-free survival. Blast expression of CD56 (> or = 20% of leukemic blasts) was seen in 16 (8%) of patients, with a median expression of 67% (range 20-99

De novo acute B cell leukemia/lymphoma with t(14;18).

The t(14;18)(q32;q21) translocation is the most common translocation in B cell malignancies being found in 80% of follicular lymphomas and about 20% of diffuse large B cell lymphomas. Only rare cases of de novo acute B cell lymphoblastic leukemia with t(14;18) have been described. We describe five cases of this entity which appears to have very homogeneous clinical, phenotypic and genotypic features. None of these patients had prior history of follicular lymphoma. The disease was characterized by acute clinical features with nodal and/or extranodal disease, massive bone marrow infiltration and

The combined treatment of Copaxone and Salirasib attenuates experimental autoimmune encephalomyelitis (EAE) in mice.

EAE is a common animal model for multiple sclerosis (MS). Immunomodulatory treatments such as glatiramer acetate (GA, Copaxone) are beneficial in EAE but are not universally effective in the clinic. The Ras inhibitor farnesylthiosalycylic acid (FTS, Salirasib), efficiently ameliorate EAE as well. Here we demonstrate a synergistic beneficial effect of the combined GA and FTS treatment on EAE; 22.5% of the combined-treatment mice developed disease compared to 87.5%, 77.5% and 82.5% of mice treated with vehicle, GA and FTS, respectively, results supported by MRI, histological, immunological and b

Differentiation Syndrome Associated With Enasidenib, a Selective Inhibitor of Mutant Isocitrate Dehydrogenase 2: Analysis of a Phase 1/2 Study.

IMPORTANCE: Enasidenib mesylate, a mutant isocitrate dehydrogenase 2 (IDH2) protein inhibitor that promotes differentiation of leukemic myeloblasts, was recently approved by the US Food and Drug Administration for use in relapsed/refractory (R/R) mutant IDH2 acute myeloid leukemia (AML). During the first study of enasidenib in humans, a minority of patients with advanced myeloid neoplasms experienced unexpected signs/symptoms of a differentiation syndrome (DS), a potentially lethal entity. OBJECTIVE: To characterize IDH-inhibitor-associated DS (IDH-DS) and its effective management. DESIGN, S

Molecular detection of minimal residual disease is associated with early relapse in adult acute lymphoblastic leukemia.

Several studies in childhood acute lymphoblastic leukemia (ALL) have documented that molecular detection of minimal residual disease (MRD) based on screening for T-cell receptor and immunoglobulin gene rearrangements can identify patients at a high risk of relapse. In our experience, evaluation of MRD in adult ALL can help to identify high risk patients.

Myelodysplasia and the leukemias.

The armistice after World War II marked the beginning of an era that was to last to the end of the present century. It was an era in which many changes in medicine and nursing combined to alter the entire philosophy of managing malignant disease. More specifically, the fluid-phase tumors, which comprise myelodysplasia and the leukemias, were singled out for special attention. First there was the ease with which blood and bone marrow could be sampled, making serial investigations simple and practical. Second, cytotoxic drugs became available ranging from nitrogen mustard through cytosine arabin

Tropical spastic paraparesis and HTLV-1 associated myelopathy: clinical, epidemiological, virological and therapeutic aspects.

In 1980, Human T cell leukemia/lymphoma virus type 1 (HTLV-1) was the first oncogenic human retrovirus to be discovered. HTLV-1 belongs to the Retroviridae family, the Orthoretrovirinae subfamily and to the deltaretrovirus genus. HTLV-1 preferentially infects CD4(+) lymphoid cells in vivo. Three molecules have been identified for binding and/or entry of HTLV-1: heparan sulfate proteoglycans, neuropilin-1, and glucose transporter 1. An efficient transfer of the virus from an infected cell to a target cell can occur through the formation of a viral synapse and/or by virofilm structure. As for al

Cutaneous T cell lymphoma: the helping hand of dendritic cells.

Since its introduction 25 years ago, cutaneous T cell lymphoma has become the preferred designation for clonal malignancies of those CD4 thymus-derived lymphocytes ("cutaneous T cells") that preferentially migrate to skin. The varied cutaneous clinical presentations, dependent on the specific features of the dominant subclones of the malignant lymphocytes, historically led to confusing descriptive terms (mycosis fungoides, Sézary syndrome, lymphoma cutis, leukemia cutis, reticulum cell sarcoma of the skin). Recognition that all of these clinical presentations are cancers of a single type of ce

[Clinical and laboratory prognostic parameters for leukemic types of chronic lymphoproliferative diseases].

AIM: The aim of the study was to identify the clinical and laboratory (hematologic, biochemical and morphological) prognostic parameters of chronic leukemic lymphoproliferative diseases (CLLPD). METHODS: The study included 155 CLLPD patients. Analysis was performed in the overall CLLPD population and separately in a subgroup of patients with B chronic lymphocytic leukemia with variants (B-CLL+V) including typical B chronic lymphocytic leukemia (B-CLL), mixed chronic lymphocytic leukemia and prolymphocytic leukemia (CLL/PLL), and a variant of chronic lymphocytic leukemia with lymphoplasmocytoi

Ocular Manifestations Associated with Hematologic Malignancies: Mechanisms, Diagnosis, and Management.

Background: Hematologic malignancies (HMs), including leukemia and lymphoma, are systemic diseases that may cause a wide range of ocular manifestations. Methods: We searched PubMed/MEDLINE (2015-2026) and identified articles with an emphasis on clinically relevant studies and recent developments. Results: Clinically, ocular involvement presents with diverse manifestations, including retinal hemorrhage, vitreoretinal lymphoma, choroidal infiltration, orbital masses, treatment-related ocular toxicities, graft-versus-host disease, and secondary infectious complications. These findings may mimic o

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… , diplopia, orbital mass, chloroma | Extramedullary leukemic infiltration | Less common | MRI, CT, B-scan, biopsy | Timely initiation of chemotherapy | [ 18 , 19 , 20 ] Anterior Segment | Ocular hypertension, conjunctival infiltration, iris lesions | Leukemic infiltration impairs aqueous humor outflow … This treatment regimen can alleviate ocular inflammatory responses and reduce leukemic infiltration [ 151 ]. …

The Bone Marrow Microenvironment Mechanisms in Acute Myeloid Leukemia.

Bone marrow (BM) is a highly complex tissue that provides important regulatory signals to orchestrate hematopoiesis. Resident and transient cells occupy and interact with some well characterized niches to produce molecular and cellular mechanisms that interfere with differentiation, migration, survival, and proliferation in this microenvironment. The acute myeloid leukemia (AML), the most common and severe hematological neoplasm in adults, arises and develop in the BM. The osteoblastic, vascular, and reticular niches provide surface co-receptors, soluble factors, cytokines, and chemokines that

Cardiac Involvement in Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma.

Cardiac involvement of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is recognized extremely rarely. In addition, most CLL/SLL patients with heart infiltration are asymptomatic. In this review, we present the results of a literature search for English language articles concerning CLL/SLL or Richter transformation with symptomatic cardiac involvement. In total, 18 well-described cases with CLL/SLL and heart infiltration were identified. Only three patients were not diagnosed with CLL/SLL before the cardiac manifestation. In other patients, cardiac CLL/SLL was diagnosed betwe

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… Schwatz and Shamsuddin identified leukemic infiltrates in spleen and lymph nodes in histologic sections in 47 patients with CLL [ 8 ]. The liver was involved in 98 per cent of cases, kidney in 90 per cent, adrenal gland in 71 per cent and heart in 64 per cent. … Assiri 2005 [ 31 ] | 83/M | CLL diagnosed 10 years before cardiac manifestation | Acute myocardial infarction, postmortem examination showed endocardial fibroelastosis and leukemic infiltration of the endocardium, myocardium, and coronary arteries | Resuscitation | The patient died despite resuscitation …

Silencing SFRP1 in bone mesenchymal stem cells alleviates pediatric B-ALL-driven bone loss by activating Wnt/β-catenin signaling.

BackgroundBone loss is the most common skeletal complication of childhood acute lymphoblastic leukemia (ALL) and seriously affects the long-term survival quality of children. However, the mechanisms behind bone loss are complicated and need to be elucidated. This study seeks to examine the principal parameters influencing the osteogenic development of bone marrow mesenchymal stem cells (BMSCs) in pediatric patients with B-cell acute lymphoblastic leukemia (B-ALL) experiencing bone loss, and to identify viable ways for alleviating bone loss.MethodsFirstly, bone mass of adolescent B-ALL patients

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… Bone loss in ALL results from two converging mechanisms: direct leukemic infiltration that disrupts bone remodeling, and chemotherapy-induced toxicity—especially from glucocorticoids—which suppress osteoblast (OB) activity and enhance osteoclastogenesis [ 4 , 5 ]. … Fig. 7 Discussion The pathogenesis of bone loss in pediatric B-ALL represents a multifactorial process involving leukemic infiltration, microenvironmental remodeling, and dysregulated bone metabolism. …

Leukemia Cutis Associated with Secondary Plasma Cell Leukemia.

Plasma cell leukemia is an uncommon, aggressive variant of leukemia that may occur de novo or in association with multiple myeloma. Leukemia cutis is the cutaneous manifestation of leukemia, and indicates an infiltration of the skin by malignant leukocytes or their precursors. Plasma cell leukemia cutis is a rare clinical presentation of leukemia. We present a man who developed plasma cell leukemia cutis in association with multiple myeloma. Cutaneous nodules developed on his arms and legs 50 days following an autologous stem cell transplant. Histopathologic examination showed CD138-positive n

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… Interestingly, cutaneous leukemic infiltrations have been observed to occur in locally immunocompromised cutaneous areas, secondary to burns, herpes zoster viral infections, intramuscular injections, recent surgeries, and trauma [ 4 , 8 ]. …

Overcoming resistance to immune checkpoint inhibitor therapy in acute myeloid leukemia.

Acute myeloid leukemia (AML) is characterized by molecular, immunological, and clinical heterogeneity and this has been reflected with changing paradigms in the treatment landscape of AML in recent years. Despite high remission rates with conventional chemotherapy regimens, most patients eventually relapse and have disappointing outcomes in the relapsed setting, with estimated 5-year overall survival rates ranging from 10-15%. Immune checkpoint inhibitors (ICIs) restrain T-cell suppressive signals delivered through cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) or programmed cell death p

Acute myeloid leukemia with plasmacytoid dendritic cell proliferation: A case report and literature review.

The present study describes a rare case of plasmacytoid dendritic cell-associated acute myeloid leukemia (pDC-AML). A 70-year-old male patient was diagnosed with pDC-AML and underwent induction chemotherapy using the venetoclax + azacitidine (VA) regimen. After 3 weeks of treatment, bone marrow examination indicated a morphologic leukemia-free state (MLFS); however, the patient experienced persistent cytopenia, which was further complicated by severe pneumonia and gastrointestinal bleeding, both of which improved following treatment. After 3 weeks in MLFS, bone marrow morphology and minimal re

Updates in novel immunotherapeutic strategies for relapsed/refractory AML.

Acute myeloid leukemia (AML) is a severe hematological malignancy with poor outcomes, particularly in older adults. Traditional treatment options like high-dose chemotherapy often lead to refractory or relapsed AML, with even worse outcomes. New therapies for relapsed and refractory AML are needed, and this review explores the most recent advancements in immunotherapy in AML. Checkpoint Inhibitors utilizing innate or adaptive immune targeting have shown potential to improve AML outcomes when combined with hypomethylating agents and chemotherapy. The use of adoptive cell therapy in AML demonstr

Systemic Mastocytosis with Smoldering Multiple Myeloma: Report of a Case.

Systemic mastocytosis (SM) is a disease characterized by a clonal infiltration of mast cells affecting various tissues of the body. It is grouped into six different subtypes according to the World Health Organization classification. It is called indolent systemic mastocytosis (ISM) when there is no evidence of end organ dysfunction, while the presence of end organ dysfunction defines aggressive systemic mastocytosis (ASM). When SM coexists with a clonal hematological disorder, it is classified as systemic mastocytosis with associated clonal hematological nonmast cell lineage disease (SM-AHNMD)