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[Clinico-morphological characteristics of status thymicolymphaticus in adolescents and adults].

Analysis of 18 autopsy observations of status thymicolymphaticus (STL) in patients aged from 14 to 44 years is presented. The clinical diagnosis of STL in adults was shown to be unsatisfactory because physicians are not properly acquainted with its clinical manifestations. The latter are in some cases conditioned by predominant involvement of the endocrine system, in others by incompetence of immunogenesis. Fatal complications developing in some cases of STL are associated with congenital abnormalities in the cardiovascular system. Morphological signs of STL include hyperplasia of the thymus a

Unicentric Pulmonary Castleman Disease Mimicking Lung Cancer: A Case Report.

Castleman disease (CD) is a rare lymphoproliferative disorder typically involving mediastinal lymph nodes. Pulmonary involvement without mediastinal lymphadenopathy is extremely rare and often mimics lung cancer. A 76-year-old man presented with an incidental left upper lobe opacity that progressed over 3 years to a 29 mm heterogeneous enhancing nodule. Surgical resection revealed hyaline vascular unicentric CD with characteristic pathology. The patient remained disease-free at 24 months. Pulmonary CD should be considered in enlarging lung nodules with enhancement and no mediastinal lymphadeno

Gamma heavy chain disease treated with daratumumab-based regimen: a first case report and review of literature.

We report on the use of a daratumumab-CHOP regimen for treatment of gamma heavy chain disease (γHCD) in a 79-year-old woman. γHCD is a very rare hematological disease, often associated with an underlying lymphoproliferative disorder. Only a few cases are reported in the literature, and, therefore, strong evidence is lacking regarding new therapeutic strategies. We attempted a treatment with a monoclonal anti-CD38 antibody in association with conventional chemothorapy, due to CD38 expression by clonal cells. This is the first reported case in the literature, in a disease in which very few novel

Recurrent Pleural Effusion Associated With Candida Albicans Infection in Transplanted Kidney: A Case Report.

Recurrent pleural effusions are associated with significant morbidity and mortality. Pleural effusions are frequently seen in patients with chronic renal failure due to fluid retention. Pleural effusions in renal transplant patients are usually related to secondary pulmonary infections, surgical complications, drug toxicities, or post-transplant lymphoproliferative disorder (PTLD). We describe an unusual cause of recurrent pleural effusion attributed to fungal infection in a transplanted kidney due to activation of the renin-angiotensin-aldosterone system (RAAS), successfully treated with anti

Pulmonary Nodular Lymphoid Hyperplasia.

Pulmonary nodular lymphoid hyperplasia is an uncommon reactive lymphoproliferative disorder that presents as an asymptomatic lung mass. The histopathologic diagnosis of pulmonary nodular lymphoid hyperplasia may be challenging because of its morphologic overlap with other diseases, such as extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue and immunoglobulin G4-related sclerosing disease. Despite the similarities, there are distinctive morphologic and phenotypic features that allow for the correct diagnosis in the majority of cases. This review aims to discuss the clinicopa

Primary cutaneous diffuse large B-cell lymphoma presenting as chronic non-healing ulcer.

Primary cutaneous diffuse large B-cell lymphoma is an uncommon and aggressive lymphoproliferative disorder with a rapid growth rate and dismal prognosis. We present the case of a 91-year-old female with an unusual manifestation of primary cutaneous diffuse large B-cell lymphoma, mimicking other more prevalent diseases like chronic non-healing venous ulceration. Dermatopathologic evaluation rendered the correct diagnosis. A discussion of this rare presentation is important for clinician consideration to prevent misdiagnosis and prolongation of proper management in patients with chronic non-heal

[Cryoglobulinemia in a case of multiple myeloma associated with viral hepatitis B].

Cryoglobulinemia is a biochemical disorder accompanying many infectious, systemic, and lymphoproliferative conditions. Cryoglobulins are proteins capable of reversible precipitation or gel formation at temperatures below 37 degrees C. There are 3 types of cryoglobulins depending on the type of immunoglobulins in their composition; hence, three types of cryoglobulinemia occurring in different diseases. Clinical manifestations of cryoglobulinemia are sometimes inapparent, various combinations of the symptoms of the disease frequently occur. An example of atypical acute cryoglobulinemic vasculiti

Reversed halo sign in lymphomatoid granulomatosis.

Lymphomatoid granulomatosis is a rare lymphoproliferative disorder which affects extranodal sites, most commonly lung. Radiologically, it typically presents with multiple nodular opacities that may wax and wane. The reversed halo sign has previously been reported in cryptogenic organizing pneumonia and more recently in South American blastomycosis. We describe a case of histologically proven lymphomatoid granulomatosis in a patient who presented initially with the more typical nodular opacities, which subsequently progressed into the reversed halo sign. To the best of our knowledge, this assoc

Schnitzler syndrome associated with systemic marginal zone B-cell lymphoma.

Schnitzler syndrome is a rare condition defined by chronic urticaria and monoclonal IgM gammopathy. Malignant evolution to Waldenström disease (WD) has been reported in several cases. We report a 49-year-old man who developed a marginal zone B-cell lymphoma (MZL) 3 years after the beginning of Schnitzler syndrome. This is the first report of MZL in association with Schnitzler syndrome. IgM gammopathy is a clue for the diagnosis of Schnitzler syndrome. This condition needs to be closely monitored; it can precede the onset of an authentic lymphoproliferative disorder including WD and rarely MZL.

Paraneoplastic pemphigus and bronchiolitis obliterans associated with a mediastinal mass: A rare case of Castleman's disease with respiratory failure requiring lung transplantation.

Castleman's disease is an infrequent and usually benign lymphoproliferative disorder. Resection of the tumor usually is curative. The immunostimulatory nature of the tumor can, in rare instances, result in paraneoplastic manifestations. The authors present a case of a 14 year old with mucocutaneous ulcerations and progressive dyspnea that was found to have a large mediastinal mass and circulating autoantibodies that were responsible for his paraneoplastic pemphigus and bronchiolitis obliterans. In spite of aggressive immunotherapy to control the autoimmune mucocutaneous lesions, the pulmonary

Hairy cell leukemia variant with t(2;8)(p12;q24) abnormality.

Hairy cell leukemia variant is an uncommon chronic B-cell lymphoproliferative disorder characterized clinically by splenomegaly and marked leukocytosis. Cytologically, the leukemic cells are distinguishable from those of classical hairy cell leukemia by the presence of single, central, and vesicular nucleoli. Cytogenetic information for this uncommon leukemia is scanty, although structural abnormalities involving 7q34 have been reported in few cases. We report a patient with hairy cell leukemia variant who has t(2;8)(p12;q34) but without [corrected] c-MYC oncogene rearrangement.

Recent advances on Epstein-Barr virus infectious mononucleosis.

Infectious mononucleosis (IM) is an acute, self-limited lymphoproliferative disorder caused by EBV. The classic features consist of fever, malaise, easy fatigability, pharyngotonsillitis, cervical lymphadenopathy, splenomegaly, subclinical hepatitis, and atypical lymphocytosis. Symptomatic IM occurs in older children and adolescents, whereas the subclinical IM is the rule in toddlers and young children. In general the prognosis is good, even in the more prolonged and serious cases. The laboratory diagnosis relies on the demonstration of heterophil antibody in the serum. Since there is no effec

A Rare Case of Autoimmune Hemolytic Anemia in Pancreatic Adenocarcinoma.

Autoimmune hemolytic anemia (AIHA) is the immune-mediated destruction of red blood cells leading to anemia. It is a well-known paraneoplastic syndrome in hematological malignancies, particularly lymphoproliferative disorders but rarely reported in solid tumors. In this report, we describe the case of a 79-year-old gentleman who presented with mixed AIHA, initially treated with methylprednisolone and rituximab, resulting in laboratory improvement. CT abdomen and pelvis showed a 3.6 cm pancreatic tail mass concerning for neoplasm with splenic vein thrombosis and carcinomatosis. The biopsy reveal…

Methotrexate-Associated Systemic Lymphadenopathy Exhibiting a Marked Elevation of the Serum IgG4 Concentrations.

Methotrexate (MTX) is an established anchor drug used for the treatment of rheumatoid arthritis. Patients receiving MTX are at an increased risk of developing MTX-associated lymphoproliferative disorders (MTX-LPDs). A diagnosis of MTX-LPD requires a pathological evaluation; however, biopsy samples are often unavailable because of the anatomical location of the lesions and their rapid regression following MTX withdrawal. In cases in which a biopsy is not feasible, biomarkers may aid in predicting the responsiveness to MTX cessation. This report describes a case of MTX-associated systemic lympha…

Платето-сателлитизм у пациента с подземным заражением, Иммун Тромбоцитопенитический Purpura (ITP) и Множественный склероз.

Платейный сателлитизм (PS) — явление витро тромбоцитов, прилипающих вокруг белых кровяных клеток, особенно в образцах крови, антикоагуляции с K3EDTA. В некоторых случаях это может привести к ложной тромбоцитопенсии, без дисфункции тромбоцитов или кровотечения. Диагноз делается путем периферического анализа мазка крови. Потенциальный механизм для PS по-прежнему в значительной степени неизвестен; однако он, возможно, предусматривает формирование антител IgG против рецептора IIb/IIIa (GPIIb/IIIa) из тромбоцита. PS наблюдается в различных медицинских условиях, включая инфекционные, аутоиммунные и

Совпадение в аннотации оригинала
Platelet satellitism (PS) is an in vitro phenomenon of platelets adhering around white blood cells, especially in blood samples anticoagulat … Ib/IIIa). PS has been observed in various medical conditions, including infectious, autoimmune, and lymphoproliferative disorders, without an obvious causative relationship. Here, we describe a case of PS in a patient who presented with infection in the setting of underlying Immune Thombocytopenic Purpura and Multiple Sclerosis.

Cold Agglutinin Disease: A Rare Paraneoplastic Manifestation of a Thyroid Malignancy.

A paraneoplastic syndrome is the presence of signs and symptoms due to cancer, but it is not a consequence of the mass effect of a tumour. It typically occurs in middle-aged to older patients with solid tumors (lung, breast, and ovaries), and hematological malignancies (leukemia and lymphoma). Autoimmune hemolytic anaemia is also a well-defined paraneoplastic phenomenon in lymphoproliferative disorders and rare solid tumour malignancies such as renal cell carcinoma, ovarian dermoid cysts, thymus cell cancer, Kaposi sarcoma, and cancers of the breast, pancreas, thyroid, and prostate. Most of th…

Multicentric Castleman Disease and Concurrent Hematological Disorders: The Occurrence of Plasmacytoma and the Hypotheses Arising from Literature Review.

The concomitant presence of Castleman disease (CD) with other hematological pathology is an event described in the literature with increasing frequency, able to modify the diagnostic and curative approach in such patients. Very few studies in the literature describe the association of CD with concomitant neoplastic diseases; the most frequent are Kaposi's sarcomas (especially in HIV and human herpes virus-8-positive patients) and lymphoproliferative disorders, such as lymphomas. Instead, since the association with plasma cell diseases such as multiple myeloma and plasmacytoma is infrequent, th…

A case of primary cutaneous anaplastic large cell lymphoma on eyelid.

Primary cutaneous anaplastic large cell lymphoma is a kind of cluster of differentiation 30+ primary cutaneous lymphoproliferative disorders with a relatively good prognosis in the absence of high-stage disease. Primary cutaneous anaplastic large cell lymphoma shows a higher frequency in males and commonly affects the head and neck. Palpebral involvement is very rare. We present a 42-year-old lady patient with primary cutaneous anaplastic large cell lymphoma involving the eyelid which was initially misdiagnosed as stye. The patient underwent a total excision of the lesion and showed complete r…

Epstein-Barr virus encephalitis in a patient with rheumatoid arthritis.

A 53-year-old woman with a 6-year history of rheumatoid arthritis (RA) presented with pharyngeal pain, fever, and altered mental status. The … tis due to elevated levels of EBV-DNA in the cerebrospinal fluid and serum. Although MTX-associated lymphoproliferative disorders are well-known complications in patients with RA, EBV encephalitis requires careful attention for such patients undergoing treatment with multiple potent immunosuppressants.

Deep Sequencing of Immunoglobulin Genes Identifies a Very Low Percentage of Monoclonal B Cells in Primary Cutaneous Marginal Zone Lymphomas with CD30-Positive Hodgkin/Reed-Sternberg-like Cells.

The spectrum of cutaneous CD30-positive lymphoproliferative disorders encompasses both inflammatory and neoplastic conditions. CD30+ Hodgkin and Reed-Sternberg-like cells have been occasionally reported in primary cutaneous marginal zone lymphoma, where they are thought to represent a side neoplastic component within a dominant background of lymphomatous small B cells. Herein, we describe the histological and molecular findings of three cases of primary cutaneous marginal zone lymphomas with CD30+ H/RS cells, in which next-generation sequencing analysis revealed the clonal population to consis…