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Y-Linked Pseudoautosomal Hodgkin Lymphoma
Y-Linked Pseudoautosomal Hodgkin Disease

Presentation

Nodular sclerosis subtype affects young adults and presents with early disease stage, while mixed cellularity HL is prevalent in both children and elderly patients and commonly presents with advanced disease stage. [ncbi.nlm.nih.gov]

Case presentation In Maya healthy young male of 32 years old presented for dry cough and dyspnea associated with fever over the past two months. [transroute.ro]

[…] cytoplasm and pyknotic reddish nuclei "Lacunar" cells: HRS cells surrounded by formalin retraction artifact, characteristic for nodular sclerosing subtype Neoplastic cells are 0.1 to 10% of cellular infiltrate Rich inflammatory / reactive background is present [pathologyoutlines.com]

Radiology 68: 845–848Google Scholar Sandhu KS, Cohen H, Radin R, Buck FS (1987) Blue rubber bleb nevus syndrome presenting with recurrences. [link.springer.com]

Workup

Overall, a comprehensive workup is essential for both diagnosis and staging of Hodgkin lymphoma. [ncbi.nlm.nih.gov]

Treatment

The initial treatment of Hodgkin lymphoma depends on subgroup treatment. [ncbi.nlm.nih.gov]

In the early s it gave ample coverage to a critical hodgkin cancer curable from Heidelberg epidemiologist Ulrich Abel, PhD, showing that chemotherapy was ineffective in the treatment of advanced carcinomas. [asspub.ro]

It is a curable disease that affects predominantly young patients years oldwith a generally good prognosis if a complete response to chemotherapy is obtained after the first-line treatment. [transroute.ro]

New recommendations are given regarding treatment with BEACOPPescalated, brentuximab vedotin and anti-PD-1 antibodies. [esmo.org]

[…] pruritus (by some authors); associated with stage 3 or 4, mixed cellularity and lymphocyte depleted subtypes Pain in lymph nodes may occur with alcohol consumption (paraneoplastic symptom) Most patients have cutaneous anergy that persists even after treatment [pathologyoutlines.com]

Prognosis

Prognosis depends on several prognostic factors, including disease stage. [ncbi.nlm.nih.gov]

[…] evidence of disease elsewhere for at least 6 months Involvement of regional lymph nodes does not exclude diagnosis (Joint Bone Spine 2000;67:446) Symptoms: bone pain, pathologic fracture, mass, B symptoms; neurologic symptoms if vertebral involvement Prognosis [pathologyoutlines.com]

It is a curable disease that affects predominantly young patients years oldwith a generally good prognosis if a complete response to chemotherapy is obtained after the first-line treatment. [transroute.ro]

Ultraschall 4: 47–48Google Scholar Thompson GB, Van Heerden JA, Martin JK, Schutt AJ, Ilstrup DM, Carney JA (1985) Carcinoid tumors of GI tract: presentation, management, and prognosis. [link.springer.com]

Etiology

Hodgkin lymphoma has an excellent overall prognosis with approximately an 80% cure rate.[1][2][3] Etiology The exact etiology of Hodgkin lymphoma is unknown. [ncbi.nlm.nih.gov]

[…] population Expression of FasL in most HRS cells plus loss of FasL expression in the follicular dendritic cells (FDC) of the germinal center suggests a disturbed FDC microenvironment that may contribute to its pathogenesis (Am J Surg Pathol 2001;25:388) Etiology [pathologyoutlines.com]

Epidemiology

[…] subtypes: lymphocyte rich CHL (LRCHL), nodular sclerosis CHL (NSCHL), mixed cellularity CHL (MCCHL) and lymphocyte depleted CHL (LDCHL) The 4 subtypes have identical immunophenotypic and genetic features but different clinical features and EBV association Epidemiology [pathologyoutlines.com]

Epidemiology, outcome, targeted agents and immunotherapy in adolescent and young adult non-Hodgkin and Hodgkin lymphoma. Br. J. Haematol. 2019 Jun;185(6):1142-1157. [PubMed: 30729493] 4. PDQ Pediatric Treatment Editorial Board. [ncbi.nlm.nih.gov]

Pathophysiology

[…] also rare Localized disease (stage I & II) in > 60% of patients Mediastinal involvement in ~60% (mostly nodular sclerosing subtype) Splenic involvement: 20%, bone marrow: 5% Splenic and abdominal involvement more frequent in mixed cellularity subtype Pathophysiology [pathologyoutlines.com]

Pathophysiology Hodgkin lymphoma has unique neoplastic cells in both the classical and NLP-HL types. Reed-Sternberg (RS) cell is a neoplastic, large multinucleated cell with two mirror-image nuclei (owl eyes) within a reactive cellular background. [ncbi.nlm.nih.gov]

Prevention

[…] marrow: 5% Splenic and abdominal involvement more frequent in mixed cellularity subtype Pathophysiology HRS cells are derived from germinal center B cells with "crippling" mutations of IgH variable region segment; associated with EBV in 30% of cases (may prevent [pathologyoutlines.com]

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