Primary bilateral adrenal lymphoma revealed by hemophagocytic syndrome

Alaoua, A.; Gilbert, G.; Ghannouchi, N.; Houchlef, M.; Létaief, A.; Bahri, F.

doi:10.1016/j.ando.2011.04.006

Cited by 4 publications

(1 citation statement)

References 13 publications

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“…Malignancy-associated secondary HLH is mostly described with T/NK-cell lymphomas and Hodgkin’s lymphomas, but is relatively uncommon in patients with B-cell non-Hodgkin’s lymphomas 16-19. Only one case of newly diagnosed malignant lymphoma with HLH presenting as acute hepatic failure has been described in the literature prior to this report 5.…”

Section: Discussionmentioning

confidence: 82%

Diffuse Large B-Cell Lymphoma with Secondary Hemophagocytic Lymphohistiocytosis Presenting as Acute Liver Failure

Patel

Mulvoy

et al. 2017

ACG Case Reports Journal

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Section: Discussionmentioning

confidence: 82%

Diffuse Large B-Cell Lymphoma with Secondary Hemophagocytic Lymphohistiocytosis Presenting as Acute Liver Failure

Patel

Mulvoy

et al. 2017

ACG Case Reports Journal

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Lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH): a scoping review unveils clinical and diagnostic patterns of a lymphoma subgroup with poor prognosis

Knauft,

Schenk,

Ernst

et al. 2024

Leukemia

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Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome driven by pathologic activation of cytotoxic T-lymphocytes and macrophages. Despite advances in diagnostics and management, adult patients with lymphoma-associated HLH (LA-HLH) harbor particularly poor prognosis and optimal treatment remains challenging. As systematic data on LA-HLH are scarce, we aimed to synthesize research evidence by thorough analysis of the published literature in PubMed (MEDLINE-database) within the context of a scoping review. Of 595 search results, 132 articles providing information on 542 patients were reviewed and analyzed. Median patient age was 60 years (range, 18–98) with male predominance (62.7%). B- and T-NHL were equally represented (45.6% and 45.2%), Hodgkin’s lymphoma was reported in 8.9% of the cases. The majority of patients (91.6%) presented in Ann-Arbor-Stages III and IV, and bone marrow infiltration was observed in a significant proportion of patients (61.5%). Soluble CD25 levels were markedly elevated (median 10,000 U/ml), with levels beyond 10,000 U/ml indicating unfavorable prognosis for 30-day and overall survival. 66.8% of the patients died after median 5.1 months. LA-HLH remains a clinical challenge requiring specialized management. Timely diagnosis and appropriate lymphoma-specific treatment are of utmost importance to enhance patient outcomes.

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Suspected Primary Adrenal Lymphoma (PAL) Associated With Hemophagocytic Lymphohistiocytosis (HLH)

Kim

Cheng

Jaiswal

et al. 2020

Journal of the Endocrine Society

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Adrenal incidentalomas, masses noted on imaging done for other purposes, are common with 10-15% presenting as bilateral adrenal masses. These cases can be challenging as the differential diagnosis is broad including metastatic disease, primary adrenal lymphoma (PAL), or infection, often requiring a biopsy if initial biochemical workup is unrevealing. Case description, laboratory and radiologic imaging studies and discussion of literature. A 62 year old Korean woman presents with altered mental status and fevers. She was found to have bilateral adrenal incidentalomas and retained acupuncture needles. Adrenal workup did not show biochemical evidence of hormonal excess. Infectious workup was unrevealing as was a metal/toxin work up due to retained acupuncture needles. Fevers and episodes of hypotension persisted which prevented the patient from obtaining an adrenal biopsy. Bone marrow biopsy was obtained for pancytopenia and revealed B cell lymphoma with large cell morphology and few histiocytes with hemophagocytosis, raising concern for lymphoma induced hemophagocytic lymphohistiocytosis (HLH). Primary adrenal lymphoma (PAL) associated with HLH was highly suspected in our patient given the large 7 cm bilateral adrenal masses and bone marrow biopsy findings of lymphoma. Patient was treated for diffuse large B cell lymphoma (DLBCL) with clinical improvement. PAL is a rare but aggressive lymphoma with few reported cases. It should be considered in the differential for both unilateral and bilateral adrenal masses. An early diagnosis is crucial as the main treatment is chemotherapy rather than surgery and it confers a significant survival benefit.

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Primary bilateral adrenal lymphoma revealed by hemophagocytic syndrome

Cited by 4 publications

References 13 publications

Diffuse Large B-Cell Lymphoma with Secondary Hemophagocytic Lymphohistiocytosis Presenting as Acute Liver Failure

Diffuse Large B-Cell Lymphoma with Secondary Hemophagocytic Lymphohistiocytosis Presenting as Acute Liver Failure

Lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH): a scoping review unveils clinical and diagnostic patterns of a lymphoma subgroup with poor prognosis

Suspected Primary Adrenal Lymphoma (PAL) Associated With Hemophagocytic Lymphohistiocytosis (HLH)

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