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Hemophagocytic lymphohistiocytosis in association with primary cutaneous anaplastic large cell lymphoma.

Basheer A, Padhi S, Nagarajan R, Boopathy V, Mookkappan S, Iqbal N - Case Rep Hematol (2014)

Bottom Line: Though association of HLH with systemic anaplastic large cell lymphoma (ALCL) is known, its occurrence in primary cutaneous ALCL (C-ALCL) is distinctly rare.We aim to describe a case of C-ALCL (anaplastic lymphoma kinase (ALK)-) in an elderly male who succumbed to the complication of associated HLH, which was possibly triggered by coexistent virus infection.We briefly present the literatures on lymphoma associated HLH and discuss the histopathological differentials of cutaneous CD30+ lymphoproliferative disorders.

View Article: PubMed Central - PubMed

Affiliation: Pondicherry Institute of Medical Sciences, Ganapathichettikulam, Pondicherry 605014, India.

ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) has a well known association with lymphomas, especially of T cell origin. Prognosis of lymphoma associated HLH is very poor, especially in T cell lymphomas; and, therefore, early diagnosis might alter the outcome. Though association of HLH with systemic anaplastic large cell lymphoma (ALCL) is known, its occurrence in primary cutaneous ALCL (C-ALCL) is distinctly rare. We aim to describe a case of C-ALCL (anaplastic lymphoma kinase (ALK)-) in an elderly male who succumbed to the complication of associated HLH, which was possibly triggered by coexistent virus infection. We briefly present the literatures on lymphoma associated HLH and discuss the histopathological differentials of cutaneous CD30+ lymphoproliferative disorders. We do suggest that HLH may pose diagnostic challenges in the evaluation of an underlying lymphoma and hence warrants proper evaluation for the underlying etiologies and/or triggering factors.

No MeSH data available.


Related in: MedlinePlus

Swelling measuring 2 × 2 cm over the left shoulder. Swelling was hard and non-tender.
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Related In: Results  -  Collection


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fig1: Swelling measuring 2 × 2 cm over the left shoulder. Swelling was hard and non-tender.

Mentions: On examination, he was febrile. General physical examination revealed pallor, pan-digital clubbing, and bipedal edema. Besides, there were 2 nontender, hard, subcutaneous swellings, one measuring 2 × 2 cm on the left shoulder and the other 1 × 1 cm over the left anterior axillary line (Figure 1). Systemic evaluation revealed firm, nontender hepatomegaly (liver span, 16 cm) and firm splenomegaly (4 cm below the left costal margin), and no significant peripheral lymphadenopathy. There were no stigmata of chronic liver disease. Disseminated tuberculosis, human immunodeficiency virus (HIV) infection, disseminated malignancy (lymphomas/leukemia), and connective tissue disorders were among the differential diagnoses considered.


Hemophagocytic lymphohistiocytosis in association with primary cutaneous anaplastic large cell lymphoma.

Basheer A, Padhi S, Nagarajan R, Boopathy V, Mookkappan S, Iqbal N - Case Rep Hematol (2014)

Swelling measuring 2 × 2 cm over the left shoulder. Swelling was hard and non-tender.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4227362&req=5

fig1: Swelling measuring 2 × 2 cm over the left shoulder. Swelling was hard and non-tender.
Mentions: On examination, he was febrile. General physical examination revealed pallor, pan-digital clubbing, and bipedal edema. Besides, there were 2 nontender, hard, subcutaneous swellings, one measuring 2 × 2 cm on the left shoulder and the other 1 × 1 cm over the left anterior axillary line (Figure 1). Systemic evaluation revealed firm, nontender hepatomegaly (liver span, 16 cm) and firm splenomegaly (4 cm below the left costal margin), and no significant peripheral lymphadenopathy. There were no stigmata of chronic liver disease. Disseminated tuberculosis, human immunodeficiency virus (HIV) infection, disseminated malignancy (lymphomas/leukemia), and connective tissue disorders were among the differential diagnoses considered.

Bottom Line: Though association of HLH with systemic anaplastic large cell lymphoma (ALCL) is known, its occurrence in primary cutaneous ALCL (C-ALCL) is distinctly rare.We aim to describe a case of C-ALCL (anaplastic lymphoma kinase (ALK)-) in an elderly male who succumbed to the complication of associated HLH, which was possibly triggered by coexistent virus infection.We briefly present the literatures on lymphoma associated HLH and discuss the histopathological differentials of cutaneous CD30+ lymphoproliferative disorders.

View Article: PubMed Central - PubMed

Affiliation: Pondicherry Institute of Medical Sciences, Ganapathichettikulam, Pondicherry 605014, India.

ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) has a well known association with lymphomas, especially of T cell origin. Prognosis of lymphoma associated HLH is very poor, especially in T cell lymphomas; and, therefore, early diagnosis might alter the outcome. Though association of HLH with systemic anaplastic large cell lymphoma (ALCL) is known, its occurrence in primary cutaneous ALCL (C-ALCL) is distinctly rare. We aim to describe a case of C-ALCL (anaplastic lymphoma kinase (ALK)-) in an elderly male who succumbed to the complication of associated HLH, which was possibly triggered by coexistent virus infection. We briefly present the literatures on lymphoma associated HLH and discuss the histopathological differentials of cutaneous CD30+ lymphoproliferative disorders. We do suggest that HLH may pose diagnostic challenges in the evaluation of an underlying lymphoma and hence warrants proper evaluation for the underlying etiologies and/or triggering factors.

No MeSH data available.


Related in: MedlinePlus