Indian Journal of Dermatology
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CASE REPORT
Year : 2005  |  Volume : 50  |  Issue : 4  |  Page : 227-230
Composite lymphoma: Mycosis fungoides with hodgkin's lymphoma


1 Department of Dermatology ,Lokmanya Tilak Muncipal Medical College & General Hospital, Sion, Mumbai-400022, India
2 Department of Pathology,Lokmanya Tilak Muncipal Medical College & General Hospital, Sion, Mumbai-400022, India

Correspondence Address:
Nilima Randhive
Department of Dermatology ,Lokmanya Tilak Muncipal Medical College & General Hospital, Sion, Mumbai-400022
India
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   Abstract 

Mycosis fungoides (MF) is a malignant lymphoma, primarily of the skin and is characterized by infiltration of the skin by atypical T-cells which have a tendency for epidermotropism. Hodgkins disease (HD) is considered to be a malignant lymphoma affecting predominantly the lymph nodes and characterized by presence of Reed­ Sternberg cells on histopathology, though, the exact origin of the Reed Sternberg cell and the nature of the malignant cell is not known yet. Few cases of association of mycosis fungoides with Hodgkin's lymphoma have been reported in the literature. It was reported in the past that when mycosis fungoides spreads to the lymph nodes and other viscera it frequently gets transformed into a more common lymphoma like Hodgkin's lymphoma. However it has now been proved that the two malignancies are distinct and that such patients probably have a tumour diathesis.


Keywords: Mycosis fungoides, Composite lymphoma, Hodgkin′s lymphoma


How to cite this article:
Mehta J, Phiske M, Dhurat R S, Jerajani H R, Kapoor R, Randhive N. Composite lymphoma: Mycosis fungoides with hodgkin's lymphoma. Indian J Dermatol 2005;50:227-30

How to cite this URL:
Mehta J, Phiske M, Dhurat R S, Jerajani H R, Kapoor R, Randhive N. Composite lymphoma: Mycosis fungoides with hodgkin's lymphoma. Indian J Dermatol [serial online] 2005 [cited 2014 Jul 28];50:227-30. Available from: http://www.e-ijd.org/text.asp?2005/50/4/227/19751



   Introduction Top


Mycosis fungoides limited to the skin has been associated with extracutaneous, primarily nodal, involvement by Hodgkin's lymphoma, most commonly nodular sclerosing type. About 29 cases have been reported so far.[1] The term composite lymphoma" has been proposed for the occurrence of two different histologic types of lymphoma at the same time either in one organ or two separate organs. We describe such a case with its histological and immunohistochemical characteristics.


   Case report Top


A 70-year-old male presented to us for evaluation of extremely itchy lesions all over the body since 5-6 months along with asymptomatic swellings in the left groin of one-month duration. He had lost significant weight over the past three months and complained of low-grade fever not associated with chills or rigors since one month.

On general examination, his vital parameters were normal. He had four palpable lymph nodes, 3 in the horizontal group and 1 in the vertical group in the left inguinal region. The nodes were 2-3 cm each and were discrete, firm, mobile and non-tender.

Cutaneous examination revealed generalized xerosis. There were multiple large hyperpigmented, ill-defined patches with a dry surface seen on the left side of the chest, the abdomen and on the extremities. Ichthyotic changes were noticeable on the shins and extensor aspects of both the forearms. There was no organomegaly on per abdominal examination. Complete hemogram revealed a normochromic, normocytic anemia and a raised erythrocyte sedimentation rate. He had marginally raised blood sugar levels. Rest of his biochemical profile was found to be within normal limits. Biopsy from a hyperpigmented patch on the trunk showed moderately dense lymphocytic infiltrate in the papillary dermis with evidence of atypicality of a few lymphocytes. Lymphocytes were also seen to be migrating into the epidermis at places, clinching the diagnosis of mycosis fungoides. A smear made from the buffy coat of the peripheral blood showed no atypical cells. Fine needle aspiration cytology from a left inguinal lymph node showed large atypical lymphocytes, findings suggestive of a lymphoprolifertive disorder. Hence a lymph node biopsy was performed which showed the lymphoid tissue divided into many nodules by fibrous bands with multiple large bizarre shaped multinucleate cells and a few classical  Reed-Sternberg cells More Details. These findings were consistent with type II nodular sclerosing Hodgkin's lymphoma.

Immunohistochemistry revealed the atypical cell in the skin to be of T-cell phenotype, which was CD43 and CD45RO positive, and CD20 and CD30 negative. The cells in the lymph node were found to be CD 15 and CD30 positive. X-ray chest showed ill-defined nodular opacities in the left middle zone. A CT scan of the chest revealed these nodular shadows to be unilateral and towards the centre in the parenchyma. These findings ruled out the presence of mediastinal lymph nodes or metastasis. The lesions disappeared after a course of antibiotics that confirmed their infective etiology. Ultrasonography of the abdomen showed multiple enlarged lymph nodes in inguinal region and along left iliac vessels. A plain and contrast enhanced CT of the abdomen confirmed the ultrasound findings and also revealed a hypodense lesion in the upper pole of the spleen that was suggestive of a lymphomatous deposit. Bone marrow aspiration showed uninvolved marrow.

He was started on COPP regime consisting of

Inj. Cyclophosphamide 984 mg

Inj. Vincristine 2 mg along with

Inj. Ondansetron 16 mg

as intravenous pulse on day 1 followed by

Tablet Procarbazine 150 mg once a day

Tablet Prednisolone 60 mg once a day along with

Capsule Omeprazole 20 mg once a day on days 2 to 15.

This is followed by a gap of 15 days after which the cycle is repeated 3 days after his second pulse, the patient presented to us with generalized weakness and fever. He was admitted and treated with antibiotics following which fever subsided within 2 days. All his routine investigations at that time were found to be normal. But the patient died a sudden unexplained death in the ward, the cause of which could not be ascertained as the relatives refused a post mortem examination.


   Discussion Top


Mycosis fungoides (MF) is an epidermotropic cutaneous T-cell lymphoma characterized by a proliferation of small or medium sized neoplastic T lymphocytes with cerebriform nuclei. It has an indolent clinical course with slow progression over years and in the later stages there may also be lymph node and visceral involvement. It has been widely reported in the past that when MF spreads to the lymph nodes and viscera it frequently undergoes a transformation into one of the common lymphomas such as reticulum cell sarcoma, lymphosarcoma or Hodgkin's lymphoma. [2],[3],[4]

Rappaport and Thomas in 1974 undertook a review of autopsy material from 45 well-documented cases of MF. They could find no evidence for such a transformation and they concluded "the neoplastic proliferation of MF in both cutaneous and extracutaneous tissues is distinct.[5]

Rosas-Uribe et al in 1974 reported that characteristic feature of lymph node or involvement by MF includes presence of clusters or sheets called MF cells.[6] Absence of these findings in the lymph node biopsy of our patient rules out the extracutaneous spread of MF.

Hodgkin's lymphoma (HL) is an overall uncommon form of cancer that involves primarily the lymphoid tissues. It arises almost invariably in a single node or chain of nodes and spreads characteristically to anatomically contiguous sites. The target cell of neoplastic transformation has yet to be identified with certainty.

Hodgkin's disease can manifest itself in the skin in many ways. The cutaneous lesions have been divided into two groups, non-specific, where the skin is not histologically involved and specific, where the skin is histologically involved. Specific lesions of HL include macules, patches, nodules or plaques. They may arise primarily in the skin or may be due to spread of the lymphoma from any other extracutaneous site. These lesions are characterized histopathologically by infiltrate composed predominantly of histiocytes along with eosinophils, neutrophils and scanty lymphocytes. Reed-Sternberg cells are usually evident. Absence of these findings in the skin biopsy of our patient rules out the possibility of cutaneous involvement by HL.

Block et al first described an association between MF and HL in 1963.[2] About 30 such cases have been reported since then, none of which have been from India. The most frequently reported type of HL associated with MF has been the nodular sclerosis type and in most case reports the MF precedes HL.[1] The prognosis in patients having both these malignancies has not been reported to be worse than that in patients with either malignancy alone. However old age, type II HL as compared to type I and a subdiaphragmatic disease as compared to a supradiaphragmatic disease, all of which were present in our case, carry a poor prognosis.

The exact molecular genetic relationship between MF and HL remains elusive and controversial.

Kadin, in 1985 suggested that there is a common, activated T-cell clone for lymphamatoid papulosis, MF and HL.[8]

In our patient, the immunohistochemistry showed co- existence of two different clones of malignant cells in the lymph nodes and the skin respectively.

The term "composite lymphoma" has been proposed for the occurrence of two different histologic types of lymphoma at the same time either in one organ or two separate organs.[9] The present case can be cited as an example.

 
   References Top

1.Bee CS, Blaise YP, Dunphy CH. Composite lymphoma of Hodgkin's lymphoma and mycosis fungoides: previously undescribed in the same extracutaneous site. Leukemia and lymphoma 2001;42(3):543-9.   Back to cited text no. 1    
2.Block JB, Edgcomb J, Eisen A, Vanscott ES. Mycosis fungoides: natural history and aspects of its relationship to other malignant lymphomas. Am J Med 1963; 34: 228-35.   Back to cited text no. 2    
3.Cyr DP, Geokas MC, Worseley GH. Mycosis fungoides: haematological findings and terminal course. Arch Dermatol 1966; 94:558-73.   Back to cited text no. 3    
4.Fraser JF. Mycosis fungoides: its relation to leukemia and lymphosarcoma. Arch Dermatol 1925;12:814-28.   Back to cited text no. 4    
5.Rappaport H, Thomas L. Mycosis fungoides: the pathology of extracutaneous involvement. Cancer 1974;34:1198-229.   Back to cited text no. 5    
6.Rosas-Uribe A, Variakojis D, Molnar Z, Rappaport H. Mycosis fungoides: an ultrastructural study. Cancer 1974;34:634-45.  Back to cited text no. 6    
7.Szur L, Harrison CV, Levene GM, Sarnman PD. Primary cutaneous Hodgkin's disease. The Lancet 1970;i:1016-20.   Back to cited text no. 7    
8.Kadin ME. Common activated helper-T-cell origin for lymphamatoid papulosis, mycosis fungoides and some types of Hodgkin's disease. The Lancet 1985;11:864-5.   Back to cited text no. 8    
9.Donald D, Green JA, White M. Mycosis fungoides associated with nodular sclerosing Hodgkin's disease: A case report. Cancer 1980;46:2505-8.  Back to cited text no. 9    


Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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