Cutaneous
T-Cell
Lymphoma
Mary Ellen McFadden
I
N 1806, FRENCH dermatologist Baron Jean Louis Alibert’ identified the unique characteristics of the diseaseprocessknown today as cutaneous T-cell lymphoma (CTCL). He called it “mycosis fungoides” (MF), not becauseit was a fungus, but becauseof the mushroom-like nature of the cutaneous lesions found in the advanced stagesof the disease. In 1870, Bazin’ described several clinical stages of MF with progression from a premycotic or pretumor stage to placque and nodular tumor development. A fumeur d’emble’e variant was reported by Vidal and Brocq in 18853and referred to the suddenappearanceof de novo tumors. Today, some believe that this event may signify the emergenceof a particularly aggressive neoplasmor a sudden decline in host resistance.4 Early in the 20th century, Pautrier recognized the diagnostic importance of the malignant, inflammatory cells that form characteristic band-like aggregates with the superficial dermis and later invade the epidermis as small clusters.5This cellular debris constitutes the microabcessesthat are a hallmark of MF pathology. In 1938, Sezary and Bouvrain6 characterized an unusual syndrome manifested by severe pruritus, generalized exfoliative erythroderma, circulating “monster” blood cells, and peripheral lymphadenopathy with similar atypical cells. Later, ultrastructural analysis of the abnormalcells from the skin, blood, and lymph nodes of patients with Sezary syndrome (SS) revealed their unique hyperconvoluted, cerebriform nuclei.’ Becauseof the distinct hematologic component, many investigators consider SS to be a leukemic variant of CTCL.’ However, this point is controversial, and the full implication of detecting circulating neoplastic cells has not been fully resolved. In general, CTCL patients with a high per-
From kins
The Johns
Hospital,
Mary Coordinator, Address
Hopkins
Baltimore,
Ellen
McFadden,
Oncology
Center.
The Johns
RN,
BSN,
MLA,
OCN:
The Johns Hopkins Oncology Center. reprint requests to Man, Ellen McFadden,
Johns Hopkins Oncology Center. The Johns Hopkins 600 N Wolfe St, Oncology Rm 346, Baltimore, MD Copyright 0 1991 by W.B. Saunders Company 0749-2081/91/0701-0006$5.OOlO
36
Hop-
MD. Clinical RN, Hospital, 21205.
The
centage of circulating Sezary cells have a shorter survival compared to those with a lower percentage.‘,” Sausville et al ” found that the median survival for patients with a positive peripheral blood smearwas 40 months; patients with a negative smearhad a median survival of 10 years or more. Overall, the examination of peripheralblood smearsfor the presenceof Stzary cells may be of limited value becausethese cells have also been found in the peripheral blood of patients with benign dermatoses.I2 A 1978National Cancer Institute workshop proposedthat the term cutaneousT-cell lymphoma be used to describe this entire spectrum of dermatologic disorders.4Included in this category are MF, SS, and a few other cutaneous lymphomas whose neoplastic cells show features of thymus-derived lymphocytes.’ Classically, patients with CTCL may experience a patchy superfical skin diseasefor years. Progressionis signified by the development of ulcerative tumors and the eventual spread to lymph nodesand visceral organs. BecauseMF representsthe largest segmentof the CTCL category, the terms are often usedinterchangeably. For purposes of clarity in this review, MF and CTCL should be viewed as synonymous unlessotherwise indicated. ETIOLOGY
Although the specific cause of CTCL remains obscure, recent advances in molecular biology have determined that it is a lymphoreticular neoplasmcharacterizedby the proliferation of thymusderived helper T lymphocytes.’ Approximately 80% of circulating lymphocytes are T cells. These T lymphocytes are responsiblefor cell-mediated immunity, which protects the host against intracellular organisms(viruses, fungi, parasites)and tumors, and is responsiblefor immunosurveillance and graft rejections.” CTCL can be distinguished from other lymphomasby its initial presentationin the skin, but the basic mechanismsof its evolutionary progressionare not firmly established.’ Clinical observationsappearto supportthe longheld theory that the epidermis is the initial site of neoplastic alteration. However, van Vloten and Seminars
in Oncology
Nursing,
Vol
7, No 1 (February),
1991:
pp 36-44
CUTANEOUS
T-CELL
LYMPHOMA:
NURSING
PERSPECTIVES
Willemze” summarize the results of several studies that offer another possibility. They describe new techniques in kinetic and cytologic analysis that indicate that malignant cells may arise in the lymph nodes, and then, after proliferation, migrate to other sites including the skin. If this systemic nature of the disease from the outset proves to be true, therapeutic approaches may eventually need to be altered.” Antecedent conditions such as a history of contact allergies. photosensitivity, and viral or fungal skin infections may be correlated with the development of CTCL. Increased numbers of epidermal Langerhans’ cells found in some CTCL infiltrates support the allergy theory, as these cells play a known role in the primary immune response to external contact allergens by modulating specific T lymphocytes. ‘,‘.” Whether or not a defect in their cellular mechanisms can facilitate a malignant process is debatable.x Rare cases of familial clustering have been reported, but studies to date do not confirm a common genetic basis of development.“,” Prolonged exposure to environmental toxins such as hazardous chemicals, industrial solvents, heavy metals, and drugs, including tobacco, are possible risk factors. Employment in a manufacturing occupation. especially petrochemical, textile, metal, and machinery industries, has been associated with high risk, and mortality rates from CTCL are excessive in United States counties where such industries are located. “.I5 A human T-cell leukemia-lymphoma retrovirus (HTLV- 1) has been implicated in the development of a particularly aggressive, rapidly fatal. atypical form of CTCL. Geographic viral clusters have been identified in the southeastern United States, the West Indies. parts of west Africa, and southern Japan. While the relationship between HTLV- and non-HTLV-associated CTCL is unclear, shared clinical features such as extensive skin manifestations, high leukemic cell counts, and nodal/ visceral extension strongly suggest a link.s.‘“.” Further support for the viral theory has been provided by the isolation of another related retrovirus, HTLV-V, from a CTCL patient-derived cell line.17 If other retroviruses prove to be causative agents, the current treatment of patients with CTCL will probably need to be modified. The controversy and uncertainty regarding etiology emphasizes the need for further research.
37
EPIDEMIOLOGY International incidence statistics for CTCL are unavailable because this neoplasm is frequently classified with other lymphoproliferative disorders. However, United States statistics from 1973 thru 1984 indicate that overall incidence rates doubled from 0.19 to 0.42 cases per 100,000 population. and mortality rates have risen as well. ” The incidence of CTCL is now approximately equal to or greater than that of Hodgkin’s disease.* Prevalence in this country has been estimated at 40,000 to 50,000 total cases annually.’ The disease is more common in males than females and usually occurs between 50 and 80 years of age. with a median age of 64 years, Unlike other lymphomas, CTCL is more common among blacks than whites. lx Advanced age, black race. prior malignancy, and the presence of SS at the time of diagnosis are each independently associated with a poor prognosis. “I CUTANEOUS CLINICAL MANIFESTATIONS Three distinct cutaneous clinical stages of CTCL are described: the patch, placque. and tumor stages. The patch or premycotic stage is characterized by the insidious onset of irregularly shaped, hyperpigmented patches, with or without scales. Although patch distribution may involve any body surface, common locations include the hathing trunk area, buttocks. thighs. abdomen, and breasts of females. ” Frequently, these premycotic lesions are elevated secondary to hyperplasia and to intracellular edema within the epidermis. Establishing a definitive diagnosis in this stage is often difficult as the lesions may resemble those of benign dermatoses, such as eczema, seborrhea, psoriasis, or contact dermatitis.’ Because patients may experience transient episodes of both spontaneous lesion regression and exacerbation, repeat biopsies are common. In the majority of cases, the predominant symptom is intractable pruritus, and at times, its severity may seem out of proportion to the extent of the cutaneous lesions.“’ Even in this early stage. patients need to be aware of the potentially lethal source of infection that may develop in the excoriation of such pruritic, edematous skin. The duration of this stage is usually from 2 to 5 years. but it may persist as long as 15 to 30 years.“’ As the disease progresses, indurated placques
38
MARY
may arise de novo or develop from the thickening of pre-existing patches. Well-defined Pautrier’s microabcesses can be identified extending into the epidermis, and a histologic diagnosis is now usually attainable. *i Infiltrated facial placques may produce a leonine facies resembling that seen in leprosy, while scalp involvement can result in alopecia. Nails frequently undergo dystrophic changes, and palmoplantar fissuring is common. 16320During this stage, the disease frequently begins to accelerate, and the skin lesions may become generalized and more widely distributed.’ Median survival for initial placque stage diagnosis can exceed 8 years.” In the third stage, tumors or nodules can form within the placques as well as on normal skin surfaces. Tumors can occur anywhere on the body, but are particularly common on the face, scalp, and body folds. They may vary in diameter up to 10 cm or larger, causing considerable cosmetic disfigurement. I6 Although pruritus is usually absent in this stage, a symptomatic nodule may erode and ulcerate causing severe pain, and the risk of secondary infection is significant due to the prurulent exudates that frequently develop.4 Histologic confirmation is now essentially straightforward, and patients receiving their initial diagnosis in this stage have a median survival of 40 months. ” EXTRACUTANEOUS CLINICAL MANIFESTATIONS
Because extracutaneous manifestations of CTCL are found in the majority of patients at the time of death, this aspectof the diseaseprocessis an important one to recognize. lo At initial diagnosis, patients with palpable adenopathy have a median survival of 40 months, whereasthose without such adenopathy have a median survival of more than 12 years.” Approximately 45% of patients with advanced diseasewill have palpable lymphadenopathy, with the nodes usually draining the major sitesof skin involvement. 1o*14Although the spread of CTCL from one lymph node region to another is usually centripetal, multiple nodal groups in the cervical, axillary, inguinal, and epitrochlear areasmay be enlarged. Lymphangiography is of limited diagnostic value and is no longer used for routine evaluation. It can be help-
ELLEN
MCFADDEN
ful, though, in determining the responseof patients with advanced diseaseto chemotherapy.l6 Lymph node biopsy is now recognized ashaving greater prognostic significance and is recommended for clinical staging.‘.” However, recent advancesin diagnostic technology using DNA cytophotometry, chromosomeanalysis, electron microscopy, and monoclonal antibody staining have proven that nodal spreadoccurs long before it is clinically evident. I*,** This ability to unravel the nature of CTCL at the cellular level may result in an earlier diagnosisand, thus, provide better methods for assessingthe risk:benefit ratio of various treatment modalities. Visceral extension is found in the terminal stagesof CTCL and is frequently not documented until autopsy. Patients in this poor-risk category have a median survival of 2.5 years. i ’ Commonly involved sitesinclude the liver, spleen, and lungs; but any organ system may be affected. Signs and symptoms are organ-specific, for example, hepatosplenomegaly as a result of liver-spleen metastases. Gastrointestional involvement causing massive diarrhea has been reported, and cranial nerve palsiesmay be indicative of central nervous system extension.I6 Most CTCL patients follow an indolent clinical courseand infection accountsfor nearly 50% of all deaths, primarily becauseof the lossof the normal protective skin mechanisms.’ Bacteremias, septicemias, and pneumoniasare the major infections with Staphylococcus aureus and Pseudomonas aeroginosa being the most common pathogens.14 Although gram-positive infections respondwell to appropriate antibiotic therapy, the overgrowth of gram-negative organismsis associatedwith a high mortality rate.i6 Becauseof the potential loss of intact skin barriers in even the earliest disease stage, severe morbidity and death need not be directly related to total tumor burden.” Progressivedisseminatedlymphoma in multiple organ systemsis the secondmost common causeof death. In addition, CTCL patients are prone to develop other neoplasms,including secondskin cancers, Hodgkin’s disease,and myeloid leukemia.’ Kantor et a123observed a 74% increasein second primary malignanciesafter CTCL, primarily lung and colon cancersand non-Hodgkin’s lymphoma. Such risks suggestthe value of periodic screening for early detection of secondtumors.
CUTANEOUS
T-CELL
LYMPHOMA:
DIAGNOSTIC
NURSING
PERSPECTIVES
39
STAGING
Table
2. Staging
Classification
of CTCL
Classifxation
In an attempt to stageand classify this complex disease, a CTCL workshop group proposed a tumor. node, metastases(TNM) classification system based on involvement of skin, lymph nodes, peripheral blood, and visceral organs(Tables 1 and 2). Sausville et al” presenteda modification of the staging criteria to include the classification of lymph node biopsies (Table 3), which provides more comprehensive prognostic information and may facilitate improved responsesto therapeutic intervention. A significant correlation exists between length of survival and the TNM staging classification.” Patients with stageI or IIA disease have a 90% 5-year survival, whereasthe median survival for stage IVB patients is only 2 years. Table
1. TNM
Classification
Classification
Description
T: Skin TO
Clinically and/or histopathologically suspicious lesions. Limited plaques, papules, or eczematous patches covering ~10% of the skin surface. Generalized plaques, papules, or erythematous patches covering ~10% of the skin surface. Tumor(s) (al). Generalized erythroderma.
Tl
T2
T3 T4 N: Lymph NO
nodes No clinically abnormal peripheral lymph nodes; pathology negative for CTCL. Clinically abnormal peripheral lymph nodes; pathology negative for CTCL. No clinically abnormal peripheral lymph nodes; pathology positive for CTCL. Clinically abnormal peripheral lymph nodes; pathology positive for CTCL.
Nl
N2
N3
B: Peripheral BO
Atypical present Atypical 125%).
circulating (
T-Cell
Lymphoma
Mary Ellen McFadden
I
N 1806, FRENCH dermatologist Baron Jean Louis Alibert’ identified the unique characteristics of the diseaseprocessknown today as cutaneous T-cell lymphoma (CTCL). He called it “mycosis fungoides” (MF), not becauseit was a fungus, but becauseof the mushroom-like nature of the cutaneous lesions found in the advanced stagesof the disease. In 1870, Bazin’ described several clinical stages of MF with progression from a premycotic or pretumor stage to placque and nodular tumor development. A fumeur d’emble’e variant was reported by Vidal and Brocq in 18853and referred to the suddenappearanceof de novo tumors. Today, some believe that this event may signify the emergenceof a particularly aggressive neoplasmor a sudden decline in host resistance.4 Early in the 20th century, Pautrier recognized the diagnostic importance of the malignant, inflammatory cells that form characteristic band-like aggregates with the superficial dermis and later invade the epidermis as small clusters.5This cellular debris constitutes the microabcessesthat are a hallmark of MF pathology. In 1938, Sezary and Bouvrain6 characterized an unusual syndrome manifested by severe pruritus, generalized exfoliative erythroderma, circulating “monster” blood cells, and peripheral lymphadenopathy with similar atypical cells. Later, ultrastructural analysis of the abnormalcells from the skin, blood, and lymph nodes of patients with Sezary syndrome (SS) revealed their unique hyperconvoluted, cerebriform nuclei.’ Becauseof the distinct hematologic component, many investigators consider SS to be a leukemic variant of CTCL.’ However, this point is controversial, and the full implication of detecting circulating neoplastic cells has not been fully resolved. In general, CTCL patients with a high per-
From kins
The Johns
Hospital,
Mary Coordinator, Address
Hopkins
Baltimore,
Ellen
McFadden,
Oncology
Center.
The Johns
RN,
BSN,
MLA,
OCN:
The Johns Hopkins Oncology Center. reprint requests to Man, Ellen McFadden,
Johns Hopkins Oncology Center. The Johns Hopkins 600 N Wolfe St, Oncology Rm 346, Baltimore, MD Copyright 0 1991 by W.B. Saunders Company 0749-2081/91/0701-0006$5.OOlO
36
Hop-
MD. Clinical RN, Hospital, 21205.
The
centage of circulating Sezary cells have a shorter survival compared to those with a lower percentage.‘,” Sausville et al ” found that the median survival for patients with a positive peripheral blood smearwas 40 months; patients with a negative smearhad a median survival of 10 years or more. Overall, the examination of peripheralblood smearsfor the presenceof Stzary cells may be of limited value becausethese cells have also been found in the peripheral blood of patients with benign dermatoses.I2 A 1978National Cancer Institute workshop proposedthat the term cutaneousT-cell lymphoma be used to describe this entire spectrum of dermatologic disorders.4Included in this category are MF, SS, and a few other cutaneous lymphomas whose neoplastic cells show features of thymus-derived lymphocytes.’ Classically, patients with CTCL may experience a patchy superfical skin diseasefor years. Progressionis signified by the development of ulcerative tumors and the eventual spread to lymph nodesand visceral organs. BecauseMF representsthe largest segmentof the CTCL category, the terms are often usedinterchangeably. For purposes of clarity in this review, MF and CTCL should be viewed as synonymous unlessotherwise indicated. ETIOLOGY
Although the specific cause of CTCL remains obscure, recent advances in molecular biology have determined that it is a lymphoreticular neoplasmcharacterizedby the proliferation of thymusderived helper T lymphocytes.’ Approximately 80% of circulating lymphocytes are T cells. These T lymphocytes are responsiblefor cell-mediated immunity, which protects the host against intracellular organisms(viruses, fungi, parasites)and tumors, and is responsiblefor immunosurveillance and graft rejections.” CTCL can be distinguished from other lymphomasby its initial presentationin the skin, but the basic mechanismsof its evolutionary progressionare not firmly established.’ Clinical observationsappearto supportthe longheld theory that the epidermis is the initial site of neoplastic alteration. However, van Vloten and Seminars
in Oncology
Nursing,
Vol
7, No 1 (February),
1991:
pp 36-44
CUTANEOUS
T-CELL
LYMPHOMA:
NURSING
PERSPECTIVES
Willemze” summarize the results of several studies that offer another possibility. They describe new techniques in kinetic and cytologic analysis that indicate that malignant cells may arise in the lymph nodes, and then, after proliferation, migrate to other sites including the skin. If this systemic nature of the disease from the outset proves to be true, therapeutic approaches may eventually need to be altered.” Antecedent conditions such as a history of contact allergies. photosensitivity, and viral or fungal skin infections may be correlated with the development of CTCL. Increased numbers of epidermal Langerhans’ cells found in some CTCL infiltrates support the allergy theory, as these cells play a known role in the primary immune response to external contact allergens by modulating specific T lymphocytes. ‘,‘.” Whether or not a defect in their cellular mechanisms can facilitate a malignant process is debatable.x Rare cases of familial clustering have been reported, but studies to date do not confirm a common genetic basis of development.“,” Prolonged exposure to environmental toxins such as hazardous chemicals, industrial solvents, heavy metals, and drugs, including tobacco, are possible risk factors. Employment in a manufacturing occupation. especially petrochemical, textile, metal, and machinery industries, has been associated with high risk, and mortality rates from CTCL are excessive in United States counties where such industries are located. “.I5 A human T-cell leukemia-lymphoma retrovirus (HTLV- 1) has been implicated in the development of a particularly aggressive, rapidly fatal. atypical form of CTCL. Geographic viral clusters have been identified in the southeastern United States, the West Indies. parts of west Africa, and southern Japan. While the relationship between HTLV- and non-HTLV-associated CTCL is unclear, shared clinical features such as extensive skin manifestations, high leukemic cell counts, and nodal/ visceral extension strongly suggest a link.s.‘“.” Further support for the viral theory has been provided by the isolation of another related retrovirus, HTLV-V, from a CTCL patient-derived cell line.17 If other retroviruses prove to be causative agents, the current treatment of patients with CTCL will probably need to be modified. The controversy and uncertainty regarding etiology emphasizes the need for further research.
37
EPIDEMIOLOGY International incidence statistics for CTCL are unavailable because this neoplasm is frequently classified with other lymphoproliferative disorders. However, United States statistics from 1973 thru 1984 indicate that overall incidence rates doubled from 0.19 to 0.42 cases per 100,000 population. and mortality rates have risen as well. ” The incidence of CTCL is now approximately equal to or greater than that of Hodgkin’s disease.* Prevalence in this country has been estimated at 40,000 to 50,000 total cases annually.’ The disease is more common in males than females and usually occurs between 50 and 80 years of age. with a median age of 64 years, Unlike other lymphomas, CTCL is more common among blacks than whites. lx Advanced age, black race. prior malignancy, and the presence of SS at the time of diagnosis are each independently associated with a poor prognosis. “I CUTANEOUS CLINICAL MANIFESTATIONS Three distinct cutaneous clinical stages of CTCL are described: the patch, placque. and tumor stages. The patch or premycotic stage is characterized by the insidious onset of irregularly shaped, hyperpigmented patches, with or without scales. Although patch distribution may involve any body surface, common locations include the hathing trunk area, buttocks. thighs. abdomen, and breasts of females. ” Frequently, these premycotic lesions are elevated secondary to hyperplasia and to intracellular edema within the epidermis. Establishing a definitive diagnosis in this stage is often difficult as the lesions may resemble those of benign dermatoses, such as eczema, seborrhea, psoriasis, or contact dermatitis.’ Because patients may experience transient episodes of both spontaneous lesion regression and exacerbation, repeat biopsies are common. In the majority of cases, the predominant symptom is intractable pruritus, and at times, its severity may seem out of proportion to the extent of the cutaneous lesions.“’ Even in this early stage. patients need to be aware of the potentially lethal source of infection that may develop in the excoriation of such pruritic, edematous skin. The duration of this stage is usually from 2 to 5 years. but it may persist as long as 15 to 30 years.“’ As the disease progresses, indurated placques
38
MARY
may arise de novo or develop from the thickening of pre-existing patches. Well-defined Pautrier’s microabcesses can be identified extending into the epidermis, and a histologic diagnosis is now usually attainable. *i Infiltrated facial placques may produce a leonine facies resembling that seen in leprosy, while scalp involvement can result in alopecia. Nails frequently undergo dystrophic changes, and palmoplantar fissuring is common. 16320During this stage, the disease frequently begins to accelerate, and the skin lesions may become generalized and more widely distributed.’ Median survival for initial placque stage diagnosis can exceed 8 years.” In the third stage, tumors or nodules can form within the placques as well as on normal skin surfaces. Tumors can occur anywhere on the body, but are particularly common on the face, scalp, and body folds. They may vary in diameter up to 10 cm or larger, causing considerable cosmetic disfigurement. I6 Although pruritus is usually absent in this stage, a symptomatic nodule may erode and ulcerate causing severe pain, and the risk of secondary infection is significant due to the prurulent exudates that frequently develop.4 Histologic confirmation is now essentially straightforward, and patients receiving their initial diagnosis in this stage have a median survival of 40 months. ” EXTRACUTANEOUS CLINICAL MANIFESTATIONS
Because extracutaneous manifestations of CTCL are found in the majority of patients at the time of death, this aspectof the diseaseprocessis an important one to recognize. lo At initial diagnosis, patients with palpable adenopathy have a median survival of 40 months, whereasthose without such adenopathy have a median survival of more than 12 years.” Approximately 45% of patients with advanced diseasewill have palpable lymphadenopathy, with the nodes usually draining the major sitesof skin involvement. 1o*14Although the spread of CTCL from one lymph node region to another is usually centripetal, multiple nodal groups in the cervical, axillary, inguinal, and epitrochlear areasmay be enlarged. Lymphangiography is of limited diagnostic value and is no longer used for routine evaluation. It can be help-
ELLEN
MCFADDEN
ful, though, in determining the responseof patients with advanced diseaseto chemotherapy.l6 Lymph node biopsy is now recognized ashaving greater prognostic significance and is recommended for clinical staging.‘.” However, recent advancesin diagnostic technology using DNA cytophotometry, chromosomeanalysis, electron microscopy, and monoclonal antibody staining have proven that nodal spreadoccurs long before it is clinically evident. I*,** This ability to unravel the nature of CTCL at the cellular level may result in an earlier diagnosisand, thus, provide better methods for assessingthe risk:benefit ratio of various treatment modalities. Visceral extension is found in the terminal stagesof CTCL and is frequently not documented until autopsy. Patients in this poor-risk category have a median survival of 2.5 years. i ’ Commonly involved sitesinclude the liver, spleen, and lungs; but any organ system may be affected. Signs and symptoms are organ-specific, for example, hepatosplenomegaly as a result of liver-spleen metastases. Gastrointestional involvement causing massive diarrhea has been reported, and cranial nerve palsiesmay be indicative of central nervous system extension.I6 Most CTCL patients follow an indolent clinical courseand infection accountsfor nearly 50% of all deaths, primarily becauseof the lossof the normal protective skin mechanisms.’ Bacteremias, septicemias, and pneumoniasare the major infections with Staphylococcus aureus and Pseudomonas aeroginosa being the most common pathogens.14 Although gram-positive infections respondwell to appropriate antibiotic therapy, the overgrowth of gram-negative organismsis associatedwith a high mortality rate.i6 Becauseof the potential loss of intact skin barriers in even the earliest disease stage, severe morbidity and death need not be directly related to total tumor burden.” Progressivedisseminatedlymphoma in multiple organ systemsis the secondmost common causeof death. In addition, CTCL patients are prone to develop other neoplasms,including secondskin cancers, Hodgkin’s disease,and myeloid leukemia.’ Kantor et a123observed a 74% increasein second primary malignanciesafter CTCL, primarily lung and colon cancersand non-Hodgkin’s lymphoma. Such risks suggestthe value of periodic screening for early detection of secondtumors.
CUTANEOUS
T-CELL
LYMPHOMA:
DIAGNOSTIC
NURSING
PERSPECTIVES
39
STAGING
Table
2. Staging
Classification
of CTCL
Classifxation
In an attempt to stageand classify this complex disease, a CTCL workshop group proposed a tumor. node, metastases(TNM) classification system based on involvement of skin, lymph nodes, peripheral blood, and visceral organs(Tables 1 and 2). Sausville et al” presenteda modification of the staging criteria to include the classification of lymph node biopsies (Table 3), which provides more comprehensive prognostic information and may facilitate improved responsesto therapeutic intervention. A significant correlation exists between length of survival and the TNM staging classification.” Patients with stageI or IIA disease have a 90% 5-year survival, whereasthe median survival for stage IVB patients is only 2 years. Table
1. TNM
Classification
Classification
Description
T: Skin TO
Clinically and/or histopathologically suspicious lesions. Limited plaques, papules, or eczematous patches covering ~10% of the skin surface. Generalized plaques, papules, or erythematous patches covering ~10% of the skin surface. Tumor(s) (al). Generalized erythroderma.
Tl
T2
T3 T4 N: Lymph NO
nodes No clinically abnormal peripheral lymph nodes; pathology negative for CTCL. Clinically abnormal peripheral lymph nodes; pathology negative for CTCL. No clinically abnormal peripheral lymph nodes; pathology positive for CTCL. Clinically abnormal peripheral lymph nodes; pathology positive for CTCL.
Nl
N2
N3
B: Peripheral BO
Atypical present Atypical 125%).
circulating (
