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Lymphoma presenting as Henoch-Schönlein purpura.

Schena FP, Soerjadi N, Zwi J, de Zoysa JR - Clin Kidney J (2012)

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine , North Shore Hospital, Waitemata District Health Board , Auckland , New Zealand.

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Henoch–Schönlein purpura (HSP) is a systemic vasculitis affecting the small blood vessels of the skin, the gastrointestinal tract and the kidneys... It is the most commonly seen vasculitis in childhood, the majority of cases being reported in children and in this group the condition is typically self-limiting... HSP is less common in adults and often does not present with the classic tetrad... However, when the patient was reviewed, there were a number of atypical features noted... Typically, there is neither coagulopathy, nor thrombocytopaenia, as was seen with our patient... The aetiology of HSP is unknown; however, there are often precipitating triggers, such as an infective illness, a reaction to drugs or malignancy... Malignancy has been reported as a potential trigger in patients with systemic vasculitis, and specifically in adult-onset HSP with a relative risk of 5.25 for malignancy found when compared with age-matched controls without HSP... The pathogenetic mechanisms that lead malignancy to present as HSP or any vasculitis remain poorly understood [7]... It is believed that vasculitis behaves like a paraneoplastic syndrome and several mechanisms have been proposed, and these include (i) molecular mimicry between tumour neoantigens and endothelial cells, (ii) decreased immune complex clearance, (iii) increased antibody production, (iv) dysregulated antibody class switching from IgM to IgA, (v) reduced sialylation of the IgA hinge region and (vi) increased inflammatory cytokine release during neoplastic processes... In the case presented here, a diagnosis of angioimmunoblastic T-cell lymphoma was concurrently discovered in the absence of any other identifiable trigger of HSP resulting in a prompt initiation of chemotherapy and improvement of renal function... In conclusion, we present a patient who presented with HSP but had atypical features, and who was found to have an underlying lymphoma... HSP classically presents with purpura, arthalgia, abdominal pain and renal impairment... In patients for whom there is a known history of malignancy, the development of HSP should result in re-evaluation for a new metastatic lesion... None declared.

No MeSH data available.


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The glomerulus shows diffuse mesangial hypercellularity, and a segmental proliferative lesion with neutrophils (upper left).
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SFS128F2: The glomerulus shows diffuse mesangial hypercellularity, and a segmental proliferative lesion with neutrophils (upper left).

Mentions: He was commenced on high-dose oral prednisone (100 mg daily) for haemolytic anaemia. A bone marrow biopsy was non-diagnostic. The renal biopsy light microscopy showed mesangial hypercellularity in all three glomeruli, with one glomerulus showing a segmental proliferative lesion (Figure 2). There was no scarring or interstitial inflammation. Immunofluorescene microscopy showed strong mesangial positivity for IgA, positivity for IgM and C3, and was negative for IgG and C1q. A computed tomography (CT) scan of the body demonstrated splenomegaly and cervical, thoracic and abdomino-pelvic lymphadenopathy (Figure 3). These findings prompted a right axillary node biopsy which showed typical features of angioimmunoblastic T-cell lymphoma (replacement of the nodes normal architecture with T cells, staining positive for CD3, CD4, CD10 and BCL6, with numerous high endothelial venules and follicular dendritic cells).Fig. 2.


Lymphoma presenting as Henoch-Schönlein purpura.

Schena FP, Soerjadi N, Zwi J, de Zoysa JR - Clin Kidney J (2012)

The glomerulus shows diffuse mesangial hypercellularity, and a segmental proliferative lesion with neutrophils (upper left).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400553&req=5

SFS128F2: The glomerulus shows diffuse mesangial hypercellularity, and a segmental proliferative lesion with neutrophils (upper left).
Mentions: He was commenced on high-dose oral prednisone (100 mg daily) for haemolytic anaemia. A bone marrow biopsy was non-diagnostic. The renal biopsy light microscopy showed mesangial hypercellularity in all three glomeruli, with one glomerulus showing a segmental proliferative lesion (Figure 2). There was no scarring or interstitial inflammation. Immunofluorescene microscopy showed strong mesangial positivity for IgA, positivity for IgM and C3, and was negative for IgG and C1q. A computed tomography (CT) scan of the body demonstrated splenomegaly and cervical, thoracic and abdomino-pelvic lymphadenopathy (Figure 3). These findings prompted a right axillary node biopsy which showed typical features of angioimmunoblastic T-cell lymphoma (replacement of the nodes normal architecture with T cells, staining positive for CD3, CD4, CD10 and BCL6, with numerous high endothelial venules and follicular dendritic cells).Fig. 2.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine , North Shore Hospital, Waitemata District Health Board , Auckland , New Zealand.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Henoch–Schönlein purpura (HSP) is a systemic vasculitis affecting the small blood vessels of the skin, the gastrointestinal tract and the kidneys... It is the most commonly seen vasculitis in childhood, the majority of cases being reported in children and in this group the condition is typically self-limiting... HSP is less common in adults and often does not present with the classic tetrad... However, when the patient was reviewed, there were a number of atypical features noted... Typically, there is neither coagulopathy, nor thrombocytopaenia, as was seen with our patient... The aetiology of HSP is unknown; however, there are often precipitating triggers, such as an infective illness, a reaction to drugs or malignancy... Malignancy has been reported as a potential trigger in patients with systemic vasculitis, and specifically in adult-onset HSP with a relative risk of 5.25 for malignancy found when compared with age-matched controls without HSP... The pathogenetic mechanisms that lead malignancy to present as HSP or any vasculitis remain poorly understood [7]... It is believed that vasculitis behaves like a paraneoplastic syndrome and several mechanisms have been proposed, and these include (i) molecular mimicry between tumour neoantigens and endothelial cells, (ii) decreased immune complex clearance, (iii) increased antibody production, (iv) dysregulated antibody class switching from IgM to IgA, (v) reduced sialylation of the IgA hinge region and (vi) increased inflammatory cytokine release during neoplastic processes... In the case presented here, a diagnosis of angioimmunoblastic T-cell lymphoma was concurrently discovered in the absence of any other identifiable trigger of HSP resulting in a prompt initiation of chemotherapy and improvement of renal function... In conclusion, we present a patient who presented with HSP but had atypical features, and who was found to have an underlying lymphoma... HSP classically presents with purpura, arthalgia, abdominal pain and renal impairment... In patients for whom there is a known history of malignancy, the development of HSP should result in re-evaluation for a new metastatic lesion... None declared.

No MeSH data available.


Related in: MedlinePlus