Cutaneous Intravascular Lymphoma following Extravascular Lymphoma of the Lung

Abstract

Intravascular lymphoma (IL) is a rare aggressive disease characterized by exclusive or predominant accumulation of malignant lymphoid cells within the lumina of small arteries, veins and capillaries. We describe a 74-year-old woman with IL presenting in the skin as asymptomatic teleangiectatic plaques on the thighs. The cutaneous neoplasm developed 18 months after an extravascular large B cell lymphoma of the lung and heralded its relapse. No neurologic symptoms were present during the course of the fatal disease. Immunophenotypic and immunogenotypic studies confirmed a unique B cell subtype. This case of IL is unusual for the association with an extravascular B cell lymphoma suggesting a link between the two diseases.

Skin manifestations of intravascular lymphoma mimic inflammatory diseases of the skin

“…29,30,36,49,51,53,77,92 Beginning in 1986, authors increasingly included immunohistochemical data of the patients studied by them. 19,20,23,25 The majority of patients had IVL-BCL (86 patients) 31,32,34,35,[38][39][40]42,47,50,53,[55][56][57][58][60][61][62]64,67,68,73,74,77,78,80,82,83,[86][87][88][89]93,95,97 whereas a minority had IVL-TCL (33 patients). 25 In 59 patients no signs and symptoms of the skin were mentioned, and in another 75 patients authors stated specifically that the skin had been uninvolved in the lymphomatous process.…”

“…Twenty-nine had lesions restricted to the skin, 1,3,16,22,[25][26][27]40,49,50,54,57,60,68,74,75,87,89,92 whereas in the other 52 patients organs other than the skin were also involved. In 26 of these 52 patients, lesions in the skin preceded those in other organs 5,[7][8][9][10][11]13,21,24,26,30,34,37,39,40,44,48,60,61,64,66,69,93 whereas in the remaining 26, signs and symptoms of other organs preceded the appearance of lesions in the skin. 2,6,17,19,20,24,26,34,…”

“…16,21,27,30,34,39,40,60,88 Lesions were said to have involved both sides of the body in 47 patients. 1,3,5,[7][8][9]13,16,21,25,30,34,37,40,42,44,45,[48][49][50]52,54,56,58,60,61,64,68,69,72,74,82,[87][88][89]93 Most commonly lesions were described as being nodules and ⁄or plaques (49%); macules were encountered in 22AE5% of the patients. Lesions were said to be indurated or firm in 27AE5% of the patients.…”

“…37,40,57,66 45,81 Data on treatment and follow-up were available for 81 patients. [1][2][3][6][7][8][9][10][11]13,16,20,22,24,25,30,34,36,37,42,44,45,48,[50][51][52]54,[56][57][58]60,61,64,66,69,72,75,81,88,89,92,93 The vast majority of patients received combination chemotherapy such as CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone), and some received radiation treatment either as a single treatment or in combination with chemotherapy. Death from IVL that presented with lesions in the skin was explicitly mentioned in 42 patients and is summarized in Table 3.…”

Intravascular large B-cell lymphoma: Remission after rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy

“…6 The estimated 5-year survival is 50%. 18 The classification of the disease has recently been revised. The classic type of intravascular large Bcell lymphoma most commonly affects the central nervous system and skin, 19 and only rarely the bone marrow, lymph nodes, liver, or spleen.…”