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Atypical clonal T-cell proliferation in infectious mononucleosis.

An atypical case of infectious mononucleosis characterized by fever, acute tonsillitis, and bilateral cervical adenopathy is reported in a previously healthy young man. Although serology was positive for the Epstein-Barr virus, the patient did not display peripheral blood lymphocytosis or atypical, reactive lymphocytes. The patient's tonsilar tissue revealed an expanded T-zone of diffuse, monomorphous lymphocytes suggestive of lymphoma. Immunophenotypic analysis of the tonsilar tissue demonstrated more than 90% expression of pan-T markers, while pan-B markers were positive in 5-10% of the inte

Granular lymphocyte proliferative disorders: a multicenter study of 20 cases.

A series of 20 patients with granular lymphocyte proliferative disorders (GLPD) is reported. The criterion of inclusion was presence of persistent (> or = 6 months) granular lymphocytosis in the absence of any causative illness. Diagnoses made upon analytical control in half the patients of splenomegaly (25%) and hepatomegaly (25%) were infrequent. Clinical course was nonprogressive in 17/20 patients, but two developed high-grade NHL several years later and one showed progressive disease. Actuarial probability of survival at 5 years was 85%. Granular lymphocyte morphology was relatively homoge

Clinical features and splenic pathologic changes in patients with autoimmune hemolytic anemia and congenital hemolytic anemia.

We characterized the pathologic changes in 50 spleens from patients with autoimmune hemolytic anemia and in 13 spleens from patients with congenital hemolytic anemia. The major pathologic findings in autoimmune hemolytic anemia were mild to pronounced cord congestion and variable white pulp area, erythrophagocytosis in conjunction with an increased polymorphonuclear neutrophil reaction, and increased deposition of hemosiderin and extramedullary hematopoiesis. In contrast, both the severity and the frequency of polymorphonuclear neutrophil reactions, deposition of hemosiderin, and extramedullar

Immunohistology of pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica. Evidence for their interrelationship with lymphomatoid papulosis.

Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica are idiopathic, papular eruptions that exhibit certain clinicopathologic similarities to each other and to lymphomatoid papulosis. In order to determine if these disorders are also similar immunologically, we studied the immunopathology of five biopsy specimens from three cases of pityriasis lichenoides et varioliformis acuta and three biopsy specimens from three cases of pityriasis lichenoides chronica. We then compared them to our prior immunohistologic study of nine cases of lymphomatoid papulosis. Pityriasis

Persistent active Epstein-Barr virus infection and atypical lymphoproliferation. Report of two cases.

Two cases of what is apparently Epstein-Barr virus (EBV)-associated atypical lymphoproliferation which clinically suggested malignant lymphoma are discussed. Immunohistology revealed polyclonal T- and B-cell proliferation resembling progressive infectious mononucleosis. Giant cell formation and marked plasmacytosis was suggestive of Hodgkin's disease. EBV serology was initially not diagnostic, but during the course of the disease, elevated titers against early antigens and EBV-associated nuclear antigen suggested virus replication. EBV DNA was demonstrated in lymphoid cells of both cases by in

Effects of cyclosporin A on autoimmune disease in MRL/1 and BXSB mice.

MRL/1 and BXSB mice were treated daily with cyclosporin A (CyA) in an oral dose of 25 mg/kg body weight. With this dose, blood levels within the therapeutic range were obtained. In normal mice CyA in this dose significantly prolonged the survival of an H-2 incompatible skin graft, and suppressed delayed-type hypersensitivity (DTH). It had no influence on the magnitude of a primary antibody response. Autoimmune mice were treated from 6 to 22 weeks of age. CyA treatment did not alter significantly the anti-DNA and anti-IgG autoantibody levels in either strain compared with control mice, who rece

[Changes in the in vitro antibody formation in B lymphoproliferative diseases].

Peripheral blood mononuclear cells from three patients with multiple myelomas (M.M.), four patients with Essential Mixed Cryoglobulinaemia (E.M.C.) and four normal adults as controls were cultivated with or without poke weed mitogen (PWM); in vitro immunoglobulin (Ig) production was measured after seven days using the enzyme-linked immunosorbent assay method (ELISA). No significant differences in Ig production were noted between patients and controls: E.M.C. patients, on the other hand, displayed considerable spontaneous IgM production (without appreciable differences, however, between spontan

Pre-existing oncohematological disease in kidney transplant recipients: impact on graft survival, acute rejection, and long-term clinical outcomes.

INTRODUCTION: Oncohematological disorders are heterogeneous conditions that present significant challenges in management prior to transplantation. Data about rejection risk, disease recurrence, eligibility criteria, and requested remission time before kidney transplant (KT) are still lacking. METHODS: All KTRs between January 1, 2000, and March 31, 2023 (n = 2871) were analyzed. All patients with an oncohematological disease (hematological cohort, including plasma cell dyscrasias [PCDs], acute leukemia, high-grade lymphoma/post-transplant lymphoproliferative disorders [PTLDs], myeloproliferat…

Immune Dysregulation in Pediatric Common Variable Immunodeficiency: Implications for the Diagnostic Approach.

Infections and infectious complications are hallmarks of common variable immunodeficiency (CVID) and the leading cause of morbidity and mortality in affected patients at any age. However, the pediatric CVID is no longer perceived as a primary immunodeficiency associated solely with infectious manifestations; autoimmune, allergic, lymphoproliferative, and malignant disorders and organ-specific immunopathology also characterize the spectrum of non-infectious complications. In this study, we sought to determine the role of immune dysregulation and frequency of non-infectious sequelae in children

A Systematic Review on Predisposition to Lymphoid (B and T cell) Neoplasias in Patients With Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity).

Primary immunodeficiencies and immune dysregulatory disorders (PIDDs; now referred to as inborn errors in immunity) are rare disorders with a prevalence of 41. 4 or 50.5 per 100,000 persons (1). The incidence of malignancy in PIDD patents is the second-highest cause of death in children as well as adults, after infection, and is higher in certain PIDDs compared to others. We performed a systematic review of the literature to identify reports of B cell and T cell neoplasias in PIDDs and clustered them based on their classification in the IUIS schema. As would be expected, higher susceptibility

HIV-associated pruritus: etiology and management.

With the advent of highly active antiretroviral therapy (HAART), life-threatening opportunistic infection has become less common in patients … infestations, papulosquamous disorders, photodermatitis, xerosis, drug reactions, and occasionally lymphoproliferative disorders. Drug eruptions are particularly common in patients who are HIV positive, presumably as a result of immune dysregulation, altered drug metabolism, and polypharmacy. Itching can also result from systemic diseases such as chronic renal failure, liver …

Mixed cryoglobulinemia.

Mixed cryoglobulinemia (MC), type II and type III, refers to the presence of circulating cryoprecipitable immune complexes in the serum and manifests clinically by a classical triad of purpura, weakness and arthralgias. It is considered to be a rare disorder, but its true prevalence remains unknown. The disease is more common in Southern Europe than in Northern Europe or Northern America. The prevalence of 'essential' MC is reported as approximately 1:100,000 (with a female-to-male ratio 3:1), but this term is now used to refer to a minority of MC patients only. MC is characterized by variable

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… These characteristics justify the proposed term of "monotypic lymphoproliferative disorder of undetermined significance (MLDUS)" [ 3 , 12 , 25 , 59 ]. … Figure 7 Differential diagnosis between mixed cryoglobulinemia and other autoimmune-lymphoproliferative disorders in the setting of HCV infection . …

The prevalence of human T-cell leukemia virus type I infection in patients with hematologic and nonhematologic diseases in an adult T-cell leukemia-endemic area of Japan.

In order to clarify the prevalence of human T-cell leukemia virus type I (HTLV-I) infection in the Kagoshima district, Japan, a highly endem … nors was 8.5%. In In hematologic diseases, the prevalence of anti-HTLV-I was 98.3% in ATL, 28.9% in lymphoproliferative disorders except ATL, and 10.6% in myeloproliferative disorders. In nonhematologic diseases, the prevalence of anti-HTLV-I was shown to be 29.5% in pulmonary tuberculosis, 25.8% in leprosy, 33.8% in chronic renal failure (CRF), 21.9% in autoimmune diseases, a …

Multicentric Castleman disease detected during the evaluation of tongue cancer: A case report.

Castleman disease is a non-neoplastic lymphoproliferative disorder that encompasses a variety of disease types. The current study reports a case of multicentric Castleman disease that was incidentally detected during the workup for tongue cancer. A 73-year-old male visited the Tochigi Medical Center (Utsunomiya, Japan) in May 2022, with the main complaint of pain on the left side of his tongue. At his initial visit, a 25×15 mm exophytic tumor with a slightly ambiguous border was observed on the left side of the patient's tongue. Positron emission tomography/computed tomography revealed numerou

Reversible cerebral vasoconstriction syndrome in idiopathic multicentric Castleman disease under treatment with tocilizumab.

BACKGROUND: Idiopathic multicentric Castleman disease (iMCD) is a rare polyclonal lymphoproliferative disorder characterised by systemic inflammation resulting from overproduction of interleukin 6 (IL-6). While iMCD primarily affects the lymph nodes and related tissues, it can also rarely involve the central nervous system. CASE PRESENTATION: We report the case of a 58-year-old female patient with at least a 3-year history of iMCD, who experienced acute thunderclap headaches due to reversible cerebral vasoconstriction syndrome (RCVS). RCVS occurred 3 months after initiating treatment with toc

Second Malignant Neoplasms Following Treatment for Hepatoblastoma: An International Report and Review of the Literature.

Treatment intensification has improved survival in patients with hepatoblastoma (HB); however, these treatments are associated with an increased risk of late effects, including second malignant neoplasms (SMNs). Data is limited regarding SMNs following HB treatment. Cases of SMNs following treatment for HB reported in the literature and from personal communication were analyzed to further assess this late effect. Thirty-eight patients were identified. The median age at diagnosis of HB was 16 months (range: 3 to 168 mo). All patients had received a platinum agent, and almost all had anthracycli

Null T-cell phenotype mycosis fungoides with aberrant CD20 and CD56 expression: A diagnostic dilemma.

Mycosis fungoides (MF) represents the most common type of primary cutaneous T-cell lymphoma. Recognition of MF variants with divergent immunophenotypes is important for accurate diagnosis and appropriate management, as they can be confused with other lymphoma subtypes. We present a case of a 49-year-old male previously diagnosed with a cutaneous lymphoproliferative disorder with an unusual NK/T-cell phenotype. He presented with a 10-year history of pelvic girdle rash involving the right hip and upper thigh. The lesions were characterized as atrophic patches concentrated in sun-protected areas

Short stature as an initial presenting presentation of unicentric Castleman disease in a child: A case report with long-term follow-up and a literature review.

Short stature is a common clinical condition in paediatric outpatient clinics and is associated with various clinical conditions, ranging from normal variants to severe diseases. Short stature is known to be caused by chronic inflammatory conditions, in which over-produced inflammatory cytokines are reported to be involved in growth suppression. Castleman disease is a rare lymphoproliferative disorder known as a chronic inflammatory disease with overproduction of interleukin 6, which often causes systemic symptoms such as fever, fatigue, weight loss, and night sweats. Here, we report the case