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Lymphocytic aleukemic leukemia cutis
Lymphocytic aleukemic leukemia curls Shoji Taniguchi, MD, Toshio Hamada, MD, H a m o Kutsuna, MD, and Masamitsu Ishii, M D
Osaka, Japan A 21-year-old male patient had localized papules on the chest and arms. Biopsy of a lesion showed it to be a malignant lymphoid neoplasm, and immunohistochemical studies confLrmed a lymphoblastoma origin. Aberrant lymphoid cells were noted in the cutaneous blood vessels, but simultaneous examination of the peripheral blood showed no evidence of leukemia. One month elapsed before observable leukemic cells were found in the blood and sternal marrow, confirming the diagnosis of acute lymphocytic leukemia. Examination of a cutaneous biopsy specimen led to early diagnosis of the disease. (J Am Acad Dermatol 1996;35: 849-50.) W e report a case of lymphocytic aleukemic leukemia cutis. Only five such cases have been reported in the Engfish-language literature. 1-4 CASE REPORT
A 21-year-old man had an asymptomatic eruption on the chest and arms for 2 months. Examination revealed several round, erythematous to violaceous papules, 5 mm in maximal diameter, on both arms and the chest (Fig. 1). Several enlarged cervical lymph nodes were present, the largest one measuring 10 x 10 mm. A complete blood cell count showed the following values: red blood cells, 4.5 x 106/mm3; hemoglobin, 12.8 gnddl; platelets, 14.2 x 104/ram3; and white blood cells, 7,700/mm 3, with 56% segmented form, 11% band form, 26% lymphocytes, 6% monocytes, and 1% eosinophils. A biopsy specimen revealed a dense cellular infiltrate in the dermis (Fig. 2); it was composed predominantly of medium to large lymphoid, blastlike cells with an oval or lobulated nucleus (Fig. 3). Immunohistochemical studies indicated that the cells were intensely positive for CD45 (leukocyte common antigen) and CD45RO, but negative for CD20, suggesting an atypical T-cell lymphoma. A biopsy specimen of a lymph node revealed infiltration with abnormal lymphoblasts, with the same profile of surface markers. A bone marrow aspirate showed hypocellularity with ORtriO
This article is made possible through an educational grant from the Dermatological Division, Ortho Pharmaceutical Corporation. From the Department of Dermatology, Osaka City University Medical School. Reprint requests: Shoji Taniguchi, MD, Department of Dermatology, Osaka City University Medical School, 1-5-7, Asahimachi, Abenoku, Osaka 545, Japan. Copyright © 1996 by the American Academy of Dermatology, Inc. 0190-9622/96 $5.00 + 0 16/4/72960
Fig. 1. Several papules on right arm ranging between 2 and 5 mm in diameter.
leukemoid blast cells and lymphoblasts and was compatible with a diagnosis of acute lymphocytic leukemia. Chemotherapy with daunombicin was initiated. One month later, the peripheral blood was abnormal for the first time, with the following values: red blood cells, 2.3 x 106/mm3; hemoglobin, 6.3 gm/dl; platelets, 7.2 x 104/ram3; and white blood cells, 23,700/mm 3, with 7% segmented form, 1% band form, 15% lymphocytes, 7% monocytes, 4% metamyelocytes, and 66% atypical lymphocytes. A combined regimen of vincristine, prednisone, dannombicin, and L-asparaginase resulted in a marked decrease in blast cells in the sternal marrow but no regression of the skin lesions. DISCUSSION
The clinical appearance of leukemia cuffs is highly variable. In addition to specific lesions caused b y leukemic cell infiltration, there often are nonspecific lesions, in which inflammatory lesions contain no leukemic cells. 5 Aleukemic leukemia cuffs is a rare form of leukemia curls in which, initially, no 849
850
Taniguchi et al.
Journal of the American Academy of Dermatology November 1996
Fig. 2. Photomicrographofskinbiopsyspecimenshows
Fig. 3. High-power magnification demonstrates primi-
diffuse dermal infiltration with atypical cells. (Hematoxylin and eosin stain; original magnification x40.)
tive blast cell infiltrate. (Hematoxylin and eosin stain; original magnification x400.)
leukemic cells are found in the blood and in which the total white blood cell count is normal or reduced. This is an example of the rare occurrence of leukemia presenting as several asymptomatic papules in a patient with no systemic manifestations or complaints and with normal blood findings. The diagnosis o f acute lymphocytic leukemia in our patient was made by bone marrow aspiration and subsequent morphologic and cytochemical studies of blood smears. Immunohistochemistry is a valuable adjunct in histopathologic differentiation o f skin infiltrates in most cases of leukemia. 6 Ratnam et al. 7 showed that the cell type determined by a panel of antibodies and enzymes generally reflected the type o f leukemia diagnosed from bone marrow and peripheral smears. These monoclonal antibodies are also helpful in confirming the diagnosis of aleukemic leukemia cuffs.
REFERENCES 1. Dunn NL, McWilliams NB, Mohanakamlar T. Clinical and immunological correlates of leukemia cuffs in childhood. Cancer 1982;50:2049-51. 2. Yoder FW, Scuen RL. Aleukemic leukemia cuffs. Arch Dermatol 1976;112:367-9. 3. Cochrane T, Milne JA. Aleukaemic acute lymphoblastic leukaemia presenting with cutaneous lesions. Br J Dermatol 1974;91:587-9. 4. Scott O. A case of primary lymphatic leukaemia of skin. Br J Dermatol Syphilol 1951;63:371-2. 5. Su WPD. Clinical, histopathologic, and immunohistochemical correlations in leukemia cuffs. Semin Dermatol 1994; 13:223-30. 6. Buechner SA, Li CY, Su WPD. Leukemia cuffs: a histopathologic study of 42 cases. Am J Dermatopathol 1985; 7:109-19. 7. Ramam KV, Su WPD, Ziesmer SC, et al. Value of immunohistochemislry in the diagnosis of leukemia cutis: study of 54 cases using paraffin-secffon markers. J Cutan Pathol 1992; 19:193-200.
Osaka, Japan A 21-year-old male patient had localized papules on the chest and arms. Biopsy of a lesion showed it to be a malignant lymphoid neoplasm, and immunohistochemical studies confLrmed a lymphoblastoma origin. Aberrant lymphoid cells were noted in the cutaneous blood vessels, but simultaneous examination of the peripheral blood showed no evidence of leukemia. One month elapsed before observable leukemic cells were found in the blood and sternal marrow, confirming the diagnosis of acute lymphocytic leukemia. Examination of a cutaneous biopsy specimen led to early diagnosis of the disease. (J Am Acad Dermatol 1996;35: 849-50.) W e report a case of lymphocytic aleukemic leukemia cutis. Only five such cases have been reported in the Engfish-language literature. 1-4 CASE REPORT
A 21-year-old man had an asymptomatic eruption on the chest and arms for 2 months. Examination revealed several round, erythematous to violaceous papules, 5 mm in maximal diameter, on both arms and the chest (Fig. 1). Several enlarged cervical lymph nodes were present, the largest one measuring 10 x 10 mm. A complete blood cell count showed the following values: red blood cells, 4.5 x 106/mm3; hemoglobin, 12.8 gnddl; platelets, 14.2 x 104/ram3; and white blood cells, 7,700/mm 3, with 56% segmented form, 11% band form, 26% lymphocytes, 6% monocytes, and 1% eosinophils. A biopsy specimen revealed a dense cellular infiltrate in the dermis (Fig. 2); it was composed predominantly of medium to large lymphoid, blastlike cells with an oval or lobulated nucleus (Fig. 3). Immunohistochemical studies indicated that the cells were intensely positive for CD45 (leukocyte common antigen) and CD45RO, but negative for CD20, suggesting an atypical T-cell lymphoma. A biopsy specimen of a lymph node revealed infiltration with abnormal lymphoblasts, with the same profile of surface markers. A bone marrow aspirate showed hypocellularity with ORtriO
This article is made possible through an educational grant from the Dermatological Division, Ortho Pharmaceutical Corporation. From the Department of Dermatology, Osaka City University Medical School. Reprint requests: Shoji Taniguchi, MD, Department of Dermatology, Osaka City University Medical School, 1-5-7, Asahimachi, Abenoku, Osaka 545, Japan. Copyright © 1996 by the American Academy of Dermatology, Inc. 0190-9622/96 $5.00 + 0 16/4/72960
Fig. 1. Several papules on right arm ranging between 2 and 5 mm in diameter.
leukemoid blast cells and lymphoblasts and was compatible with a diagnosis of acute lymphocytic leukemia. Chemotherapy with daunombicin was initiated. One month later, the peripheral blood was abnormal for the first time, with the following values: red blood cells, 2.3 x 106/mm3; hemoglobin, 6.3 gm/dl; platelets, 7.2 x 104/ram3; and white blood cells, 23,700/mm 3, with 7% segmented form, 1% band form, 15% lymphocytes, 7% monocytes, 4% metamyelocytes, and 66% atypical lymphocytes. A combined regimen of vincristine, prednisone, dannombicin, and L-asparaginase resulted in a marked decrease in blast cells in the sternal marrow but no regression of the skin lesions. DISCUSSION
The clinical appearance of leukemia cuffs is highly variable. In addition to specific lesions caused b y leukemic cell infiltration, there often are nonspecific lesions, in which inflammatory lesions contain no leukemic cells. 5 Aleukemic leukemia cuffs is a rare form of leukemia curls in which, initially, no 849
850
Taniguchi et al.
Journal of the American Academy of Dermatology November 1996
Fig. 2. Photomicrographofskinbiopsyspecimenshows
Fig. 3. High-power magnification demonstrates primi-
diffuse dermal infiltration with atypical cells. (Hematoxylin and eosin stain; original magnification x40.)
tive blast cell infiltrate. (Hematoxylin and eosin stain; original magnification x400.)
leukemic cells are found in the blood and in which the total white blood cell count is normal or reduced. This is an example of the rare occurrence of leukemia presenting as several asymptomatic papules in a patient with no systemic manifestations or complaints and with normal blood findings. The diagnosis o f acute lymphocytic leukemia in our patient was made by bone marrow aspiration and subsequent morphologic and cytochemical studies of blood smears. Immunohistochemistry is a valuable adjunct in histopathologic differentiation o f skin infiltrates in most cases of leukemia. 6 Ratnam et al. 7 showed that the cell type determined by a panel of antibodies and enzymes generally reflected the type o f leukemia diagnosed from bone marrow and peripheral smears. These monoclonal antibodies are also helpful in confirming the diagnosis of aleukemic leukemia cuffs.
REFERENCES 1. Dunn NL, McWilliams NB, Mohanakamlar T. Clinical and immunological correlates of leukemia cuffs in childhood. Cancer 1982;50:2049-51. 2. Yoder FW, Scuen RL. Aleukemic leukemia cuffs. Arch Dermatol 1976;112:367-9. 3. Cochrane T, Milne JA. Aleukaemic acute lymphoblastic leukaemia presenting with cutaneous lesions. Br J Dermatol 1974;91:587-9. 4. Scott O. A case of primary lymphatic leukaemia of skin. Br J Dermatol Syphilol 1951;63:371-2. 5. Su WPD. Clinical, histopathologic, and immunohistochemical correlations in leukemia cuffs. Semin Dermatol 1994; 13:223-30. 6. Buechner SA, Li CY, Su WPD. Leukemia cuffs: a histopathologic study of 42 cases. Am J Dermatopathol 1985; 7:109-19. 7. Ramam KV, Su WPD, Ziesmer SC, et al. Value of immunohistochemislry in the diagnosis of leukemia cutis: study of 54 cases using paraffin-secffon markers. J Cutan Pathol 1992; 19:193-200.