Classification of Cutaneous Lymphomas in Historical Perspective

Christian A. Sander and Michael J. Flaig

Department of Dermatology, LMU, Munich, Germany

Werner Kempf and Günter Burg

Department of Dermatology, University Hospital, Zurich, Switzerland

Going back in history, classification of cutaneous lymphomas began with the introduction of mycosis fungoides by Alibert in the last century (1) and was extended by the description of the d'emblee form by Vidal and Brocq in 1885 (2).

In 1924, Letterer (3) introduced the term malignant reticulosis, which defined a proliferation of either benign or malignant reticulum cells. This term was used for decades as a collective term synonymous with malignant lymphoma (4). The cutaneous reticuloses were classified into four groups: infectious granulomas (tuberculosis, syphilis), storage diseases (xanthomatoses, histiocytosis X group), benign neoplasias of connective tissue (histiocytoma, nevoxanthoendothelioma), and malignant reticulosis (5).

With the description of Sezary syndrome by Sezary and Bouvrain in 1938 (6), another clinicopathologic entity was added to the spectrum of cutaneous lymphomas. Up until the late 1960s, cutaneous lymphomas were divided in mycosis fungoides and Sezary syndrome. For other lymphomas, the terms malignant reticu-losis or reticulum cell sarcoma were used. An extensive classification scheme including these terms was proposed by Keining and Braun-Falco in 1961 (Table 1) (7).

The next major change in lymphoma classification was the gradual linkage of cutaneous lymphomas with the classification of nodal lymphomas. For many years, malignant lymphomas had been categorized in three basic categories: Hodgkin disease, lymphosarcoma, and reticulum cell sarcoma. There was great confusion among pathologists over the use of these terms, as it was evident that other types of lymphomas existed. Thus, the necessity for a new lymphoma classification was obvious (Table 2) (8).

In 1966, Rappaport (9) proposed his classification for the tumors of the hematopoietic system. The Rappaport classification was the first classification which divided lymphomas by pattern (nodular or diffuse) and by cytologic subtypes (Table 3).

The Rappaport classification was published at a time when very little was known about our immune system. As a consequence, in the 1970s, several schemes

Table 1 Lymphoplasias of the Skin

Lymphadenosis benigna cutis (Lymphadenosis benigna cutis) Spiegler-Fendt sarcoid (Spiegler-Fendtsches Sarkoid)

Lymphocytic infiltration Jessner-Kanof (Lymphocytic infiltration Jessner-Kanof) Reticuloses (Retikulosen)

Monomorphous reticuloses (monomorphe Retikulosen) Primary reticuloses (primäre Retikulosen) Secondary reticuloses (sekundäre Retikulosen) Lipomelanotic reticulosis (lipomelanotische Retikulose) Large follicular lymphoblastoma (großfollikuläres Lymphoblastom) Senile erythroderma (Alterserythrodermie) Mastocytoses (Mastozytosen)

Granulomatous reticuloses (granulomatose Retikulosen) Mycosis fungoides (Mykosis fungoides) Hodgkin's disease (Lymphogranulomatosis maligna) Malignant tumors of the reticular connective tissue of the skin (Maligne Tumoren des reticulären Bindegewebes der Haut)

Retothelial sarcoma/retothelial sarcomatosis (Retothelsarkom/-sarkomatose) Reticulosarcomatosis (Retikulosarkomatose) Cutaneous signs in hematopoietic disorders (Hautveränderungen bei Leukosen) Chronic lymphatic leukemia (chronisch lymphatische Leukämie) Lymphosarcoma/lymphosarcomatosis (Lymphosarkom/-sarkomatose) Myeloid leukemia (myeloische Leukämie) Agranulocytosis (Agranulozytose)

such as the Kiel classification, Lukes-Collins classification, and the Working Formulation were proposed in an attempt to incorporate the highly relevant new information about the immune system (10-12). While the Working Formulation was proposed as an interim compromise approach, it became the de facto classification system in the United States. It was proposed as an alternative approach to the multitude of classification schemes in the 1970s. The categories of the Working Formulation were defined by survival data of patients treated on chemotherapy protocols used in the 1970s. The Kiel classification gained widespread use in Europe and Asia. This classification and the Lukes-Collins scheme were based on an attempt to relate the malignant lymphomas to the normal immune system. Lymphomas were diagnosed by morphology in the Working Formulation, but by a combination of morphologic and immunophenotypic criteria in the Kiel classification.

Table 2 Historical Overview of Lymphoma Classifications
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