242
Letters and Correspondence
4 . Fiveson DP. King AJ: Porphyria Cutanen Tarda in a patient with agnogenic myeloid metaplasia. Arch Derrnatol I20:538, 1984. 5 . Guyotat D, Nicolas JF. Augey D, e l al.: Porphyria CutancaTarda after allogenic bone iiiarrow transplantation for chronic myelogenous Ieuhcmia. Am J Hernatol 34:69. 1990. 6. DeRosa G, Catalano L, Selleri C . et al.: Porphyria Cutanea Tarda and hematological disea\e Am J Hernalol 29209,1988. 7 . Mukerji SJ. Primstone NR. Burn\ M, ct al.: Dual mechanism of inhibition of rat liver uroporphyrinogendecarboxilaae activity by ferrous iron; its potential role in the grnesis of Porphyria Cutanea Tarda. Gastroenterology X7:1248. 1984. 8. Kappas A. SassaS. Anderson KE: The Porphyrias. In Standbury JB. Wyngnardcn JB, Frederickson DS, Goldstein LJ. Brow M S (eds): “The Metabolic Bases of Inherited Diseasc.”Ed. 5 . New York: McCraw Hill. 1982. p. 1301. 9. Coburn PR. Ccileindn JC. Murray-Lyon IM: Porphyria Cutaned Tarda and Porphyria variegata unmasked by viral hepatitis. Clin Exp Derniatol 10:169. 1985.
VPI6. BCNU. and melphalan, 131 but only after heavy anthracycline pretreatment, in a patient with Hodgkin disease who had received complete courses of ABVD (adriamycin. bleomycin, vinblastine, dacarbazine) and VEEP (vincristine. epirubicin. VP16. prednisolone) previously. Carboplatin, BCNU, VP16. melphalan, and thiotepa, the drugs that the patient received, are individually free of significant cardiotoxicity [4]. Also, the dose of adriamycin received by the patient was smaller than the upper limit of 45@550 mgim’. beyond which severe irreversible cardiac damage occurs 141. Autologous BMT involves the administration of very high doses of chemotherapeutic agents with infusion of bone marrow to rescue the patient from the lethal hematologic toxicity. We conclude that drugs which are not otherwise cardiotoxic may cause serious cardiotoxicity when used in high doses for BMT in patients who have received heavy chemotherapy in the part. especially with the anthracycline antibiotics. Further, this cardiotoxicity may be seen even at “safe” cumulative anthracycline doses, and normal cardiac status on routine investigations prior to BMT does not preclude the possibility of serious cardiac adverse events.
JAYESH MEHTA ARNON NAGLER
Fatal Acute Cardiomyopathy Associated With “Non-Cardiotoxic” Chemotherapy for Autologous Bone Marrow Transplantation To the Editor: An I I-year-old female with Ewing’s sarcotna of the right hip in second partial remission was referred for autologous bone marrow transplantation (ABMT). In the 5 years since diagnosis. she had had a right hemipelvectoniy. and 5,000 rad irradiation each to the pelvis and the left orbit. She had received no irradiation to the chest. She had received multiagent chemotherapy at the following cumulative doses: ifosfamide 25.2 g/ m’, VP16 I.6 gim’. cyclophosphamide 8 gim’, actinornycin D 4 gim’. adriamycin 300 mpim’, and vincristine 16.7 mgim’. She had received no chemotherapy in the 2 years preceding the ABMT. During this 5 year period. 3 echocardiographic examinations, including one after cessation of all chemotherapy, were found to be normal. Immediately prior to ABMT, she was in good general condition with normal clinical cardiovascular status and a normal electrocardiogram. Echocardiogram showed normal left ventricular size (LVED 4.41 cm and LVES 3.14 cm), borderline diffuse left ventricular hypokinesis (fractional shortening 29%). and normal right ventricular function. The conditioning regimen for the ABMT included thiotepa 7.6 mg (0.2 mgikg) on days -7, - 5 . and - 3: carboplatin 575 mg (500 ingim’) on days -6 and -2; VPlh 230 mg (200 mgim’) on days -5. -3, and - I ; BCNU 345 mg (300 mgim’) on day -4;and melphalan 69 mg (60 mgim’) on days -2 and - I . The marrow was infused uneventfully on day 0. She developed tachycardia ( 140imin). gallop rhythm, hypotension (751 60 mm Hg), and tachypnea (40imin) the next day. The ECG showed sinus tachycardia and low QRS voltage. Chest roentgenogram showed interstitial infiltrates suggestive of pulmonary edema with a normal cardiac silhouette. Echocardiography showed normal left ventricular size (LVED 4.18 cm and LVES 3.44 cm), diffuse severe left ventricular hypokinesic (fractional shortening 18%). and apical akmesix. Estimated cardiac output was 0.8 L/ min, and the left ventricular ejection fraction was 2 0 6 . Biochemical investigations were unremarkable except for elevated CPK (228 Ui and CPK-MB (1748). Despite the use of inotropic agents and intensive supportive care, the cardiac function as monitored clinically and on echocardiography continued to deteriorate. ECG continued to show low voltage and no ST-T disturbances or conduction disturbances. The nadir blood counts were Hb 125 g/L. leukocytes 0. I x IO”/L, and platelets 41 X I0”iL. There war no bleeding diathesis or clinical evidence of infection. and all cultures were negative. The patient died on the 5th day after ABMT of cardiac failure. Permirsion was not granted for a postmortem examination. Cardiotoxicity is a serious adverse effect of antimalignancy cheniotherapeutic agents such as the anthracycline antibiotics 1 I and cyclophosphamide 121. Cardiac toxicity has been seen after combination therapy with
OAVlO SCHECHTER
A.J.J.T. REIN SHIMON SLAVIN Departments of Bone Marrow Transplantation and Cardiology, Hadassah University Hospital, Jerusalem, lsrael
REFERENCES I . Doroshow JH: Doxorubicin-induced cardiac toxicity. N Engl J Med 324:843-845, 1991. 2 . Goldberg M A . Antin JH. Guinan EC. Rappcport JM: Cyclophosphamide cardiotoxicity. an analysis of dosing as a risk Factor. Blood 6X:l 114-1 118. 1986. 3. Zulian G B . Selby P. Milan S. el al.: High dose melphalan. B C N U and eroposide with autologous bone inarrow transplantation for Hodgkin’s disease. Br J Cancer 59:631-635. 1989. 4. Chabner B A . Collinr JM, eds: “Cancer Chemotherapy: Principles and Practice.” Philadelphia: J.B. Lippincolt, 1990.
DermatomyositisAssociated With Acute Prornyelocytic Leukemia T o the Edimr: The association of dermatoniyositis (DM) with malignant neoplastic diseases is well known [I,2]. However. reports of DM preceding acute myelocytic leukemia are very rare. We describe a case of DM that was followed by acute promyelocytic leukemia (APL) and was moreover interestingly associated with seminoma. A 30-year-old man was admitted to our hospital because of myalgia. muscle weakness. and skin rash in April 1986. He was diagnosed as having DM by clinical signs, elevated serum muscle enzyme activity, electromyography. and skin biopsy. He was treated with high doses of prednisolone (I mgikg. daily). and improvement was seen in his clinical signs and laboratory data. However, an indurated testicular mass approximately 10 x 8 cm in size was subsequently noted. Echosonography demonstrated the image of a seminoma. He underwent orchidectomy. and histology of the testicular mass revealed seminoma. Moreover, he had a metastatic tumor infiltrating the lung, so he received four courses of combination chemotherapy (cisplatin, etoposide, peplomycin. and prednisolone). The lung mass subsequently disappeared, and his condition has remained good for 3 years.
Letters and Correspondence He presented to our institute wlth fever and general malaise in July 1990. Complete blood count showed 9 . 6 gidl, WBC 1.7 x 109/L, and platelets 85 X IO'iL. Bone marrow aspirate showed hypercellularity with 69.2% atypical promyelocytes with Auer rods. Cytochemical examination revealed that leukemic cells in the bone marrow were highly positive for peroxidase stain. Chromosomal analysis of marrow cells by G-banding revealed normal karyotype. lmrnunophenotyping of the leukemic cells was CD13 (54.8%) and CD33 (57.0%) positive. Thus, the patient was diagnosed as having APL. He was treated with behenoyl cytosine arabinoside, daunorubicin, 6-niercaptopurine, and prednisolone. A complete remission was achieved after I month, The patient remains in complete remission in June 1991. DM is a rare and severe disorder characterized by an inflammatory myopathy and typical cutaneous lesions [3]. Some reports have noted that patients with DM develop malignant neoplastic disease, and that the most common tumors associated with DM are those of the lungs. uterus, ovary, breast. prostate, and gastrointestinal tract 141. However, it is very unusual that DM was associated with APL and moreover with seminoma. T o our knowledge, this association has not been previously described. There are various possible causes for the APL seen in our patient. In this case, seminoma was treated with cytotoxic drugs. It is possible that APL occurred as a therapy-related leukemia. Therapy-related leukemias are known to occur as a side effect of treatment with cytotoxic drugs, particularly alkylating agents IS]. Moreover, therapy-related leukemias commonly accompany cytogenetic abnormality and inyelodysplastic changes [6,7]. However. in our patient the clinical presentation and evolution were not typical of therapy-related leukemias. In addition, alkylating agents were not used in this case. Thus, there was little possibility of therapy-related leukemia. Although an abnormal immune mechanism may have caused the APL, we cannot directly demonstrate it. We must also consider the possibility that the association of DM. APL. and seminoma in our patient may have been entirely coincidental. We conclude that awareness of this association may lead to earlier detection of malignancy. and hence to potentially more effective therapy.
243
Five Monoclonal Proteins in a Patient With Chronic Myelomonocytic Leukemia
To fhe Editor; Monoclonal gammopathies of undetermined significance (MGUS) constitute a heterogeneous group of diseases with a proliferation of a single clone of plasma cell homogeneous monoclonal proteins (M) without evidence of multiple myeloma, amyloidosis, rnacroglobulinemia. or other lymphoproliferative diseases [ I ] . These gammopathies can be associated with both hematologically benign and malignant diseases. as in other nonhematological processes. In approximately 2% of MGUS. biclonality can be seen 121. Isolated cases of triclonal antibodies have also been described in patients with plasma cell dyscrasia and acquired immunodeficiency syndrome (AIDS) and non-Hodgkin lymphomas 13.41. Nevertheless, as far as we know, no patient with five monoclonal proteins has even been described. This patient was diagnosed with chronic myelomonocytic leukemia on the basis of peripheral blood and bone marrow. The bone marrow karyotype shows a pathological metaphases of the 19 studied, one of them with trisomia of chromosome 8 and the other with a deletion of the short arm of chromosome 5. Blood and urine neuramidase are of 40 ngiml and 8 4 mgi24 hr respectively. Electrophoretic studies of total proteins produced the following results: total proteins 8.7 gidl albumin 4.2 gidl.. a , - G 0.5 gidl. a2-G 0.9 gidl. P-G I gidl (paraprotein p) y-G 2 . 3 gidl (paraprotein + y), and immunoglobulins IgG 1,730 nigidl, IgA 378 mgidl, and IgM 433 mgidl. High-resolution electrophoresis in agarose gel was carried out, which showed the existence of five proteins which could be identified through inmunoelectrophoresis and inmunofixation. With both techniques, two monoclonal bands corresponding to 1gG ( K and A). two monoclonal bands corresponding to IgA ( K + K), and a monoclonal band corresponding to IgM (A) were found. There was proteinuria in the urine of 15.9 mgi24 hr which was studied TOMOHIRO SUGAWARA by electrophoresis and inmunofixation. Two monoclonal bands were obKAZUYAW ENDO served IgG ( K and A) and one monoclonal band of IgA ( K ) as well as K and JUNKIMURA A free chains together with a heavy chain IgG (7). JUNNOMURA Neither chronic myelomonocytic leukemia nor monoclonal serum proKAZUMICHI FURUYAMAteins in urine proteins changed over 3 years of follow-up. This non-progresHIDEOHARIGAEsion of the disease IS an essential feature of benign monoclonal garnmopaKVOKO KANEOAthies by definition [Sl.
+
KAORU YOSHIN~GA
Second Department of lnternal Medicine, Tohoku University School of Medicine, Aobaku, Sendai, Japan
REFERENCES 1. Barnes BE: Derinatomysitis and malignancy: a review of the literature. Ann Intern Med 84:68-76. 1976.
2. Richardson JB, Callen JP: Dermatocyositis and malignancy. Med Clin North Am 73:1211-1220. 1989. 3 . Callcn JP: Dermatomyositis. Neurol Clin 5:379-403, 19x7. 4. Callen JP: Dermatomyositis: Current controversies 1987. Adv Dermatol 3:3-24. 1988. 5 . Greene MH. Boice JD. Greer BE, Blessing JA, Dembo AJ: Acute nonlymphocytic leukemia after therapy with alkylating agent5 for ovarian cancer-A study of five randomized clinical trials. N Engl J Med 307:1416-1421. 1982. 6. Michels SD, McKenna R W , Anher DC, Brunning RD: Therapy-related acute myeloid leukemia and myelodysplastic syndrome: A clinical and morphologic study of65 cases. Blood 65:136&1372. 1985. 7. Pedersen-Bjergdard J , Philip P: Cytogenetic characteristics of therapy-related acute nonlymphocytic leukaemia. preleukaeinia and acute myeloproliferative syndrome: correlation with clinical data for 61 consecutive cases. Br J Haematol
66:199-207. 1987.
A. VILLEGAS M. L ~ P ERueio Z M. ARROYO E. DEL POTRO Servicios de Hematologia y Bioquimica Hospital Universitario San Carlos Universidad Complutense de Madrid Madrid, Spain
REFERENCES I . Kyle RA: Monoclonal pammopathy of undetermined significance (MGUS): A review. Clin Haematol I1:123. 1982. 2 Kyle RA. Lust JA: Monoclonal gammopathies of undetermined significance.
Semin Hematol26:176. 1989. 3. Berg A R , Weissenburger DD. Linder J. Arnmitage JD: L)mphoplasmocytic lymphoma: Report of a case with three monoclonal proteins derived from a single neoplaatic clone. Cancer 57: 1794. 1986. 4. Ray RA, Schotters SB: Triclonal gammophathy in il patient with AIDS. Clin Chem 32:2Oc)O, 1986. 5 WaldeStriim JC: Benign monoclonal gammopathy. Acta Med Scaiid 216:435. 19x4.
Letters and Correspondence
4 . Fiveson DP. King AJ: Porphyria Cutanen Tarda in a patient with agnogenic myeloid metaplasia. Arch Derrnatol I20:538, 1984. 5 . Guyotat D, Nicolas JF. Augey D, e l al.: Porphyria CutancaTarda after allogenic bone iiiarrow transplantation for chronic myelogenous Ieuhcmia. Am J Hernatol 34:69. 1990. 6. DeRosa G, Catalano L, Selleri C . et al.: Porphyria Cutanea Tarda and hematological disea\e Am J Hernalol 29209,1988. 7 . Mukerji SJ. Primstone NR. Burn\ M, ct al.: Dual mechanism of inhibition of rat liver uroporphyrinogendecarboxilaae activity by ferrous iron; its potential role in the grnesis of Porphyria Cutanea Tarda. Gastroenterology X7:1248. 1984. 8. Kappas A. SassaS. Anderson KE: The Porphyrias. In Standbury JB. Wyngnardcn JB, Frederickson DS, Goldstein LJ. Brow M S (eds): “The Metabolic Bases of Inherited Diseasc.”Ed. 5 . New York: McCraw Hill. 1982. p. 1301. 9. Coburn PR. Ccileindn JC. Murray-Lyon IM: Porphyria Cutaned Tarda and Porphyria variegata unmasked by viral hepatitis. Clin Exp Derniatol 10:169. 1985.
VPI6. BCNU. and melphalan, 131 but only after heavy anthracycline pretreatment, in a patient with Hodgkin disease who had received complete courses of ABVD (adriamycin. bleomycin, vinblastine, dacarbazine) and VEEP (vincristine. epirubicin. VP16. prednisolone) previously. Carboplatin, BCNU, VP16. melphalan, and thiotepa, the drugs that the patient received, are individually free of significant cardiotoxicity [4]. Also, the dose of adriamycin received by the patient was smaller than the upper limit of 45@550 mgim’. beyond which severe irreversible cardiac damage occurs 141. Autologous BMT involves the administration of very high doses of chemotherapeutic agents with infusion of bone marrow to rescue the patient from the lethal hematologic toxicity. We conclude that drugs which are not otherwise cardiotoxic may cause serious cardiotoxicity when used in high doses for BMT in patients who have received heavy chemotherapy in the part. especially with the anthracycline antibiotics. Further, this cardiotoxicity may be seen even at “safe” cumulative anthracycline doses, and normal cardiac status on routine investigations prior to BMT does not preclude the possibility of serious cardiac adverse events.
JAYESH MEHTA ARNON NAGLER
Fatal Acute Cardiomyopathy Associated With “Non-Cardiotoxic” Chemotherapy for Autologous Bone Marrow Transplantation To the Editor: An I I-year-old female with Ewing’s sarcotna of the right hip in second partial remission was referred for autologous bone marrow transplantation (ABMT). In the 5 years since diagnosis. she had had a right hemipelvectoniy. and 5,000 rad irradiation each to the pelvis and the left orbit. She had received no irradiation to the chest. She had received multiagent chemotherapy at the following cumulative doses: ifosfamide 25.2 g/ m’, VP16 I.6 gim’. cyclophosphamide 8 gim’, actinornycin D 4 gim’. adriamycin 300 mpim’, and vincristine 16.7 mgim’. She had received no chemotherapy in the 2 years preceding the ABMT. During this 5 year period. 3 echocardiographic examinations, including one after cessation of all chemotherapy, were found to be normal. Immediately prior to ABMT, she was in good general condition with normal clinical cardiovascular status and a normal electrocardiogram. Echocardiogram showed normal left ventricular size (LVED 4.41 cm and LVES 3.14 cm), borderline diffuse left ventricular hypokinesis (fractional shortening 29%). and normal right ventricular function. The conditioning regimen for the ABMT included thiotepa 7.6 mg (0.2 mgikg) on days -7, - 5 . and - 3: carboplatin 575 mg (500 ingim’) on days -6 and -2; VPlh 230 mg (200 mgim’) on days -5. -3, and - I ; BCNU 345 mg (300 mgim’) on day -4;and melphalan 69 mg (60 mgim’) on days -2 and - I . The marrow was infused uneventfully on day 0. She developed tachycardia ( 140imin). gallop rhythm, hypotension (751 60 mm Hg), and tachypnea (40imin) the next day. The ECG showed sinus tachycardia and low QRS voltage. Chest roentgenogram showed interstitial infiltrates suggestive of pulmonary edema with a normal cardiac silhouette. Echocardiography showed normal left ventricular size (LVED 4.18 cm and LVES 3.44 cm), diffuse severe left ventricular hypokinesic (fractional shortening 18%). and apical akmesix. Estimated cardiac output was 0.8 L/ min, and the left ventricular ejection fraction was 2 0 6 . Biochemical investigations were unremarkable except for elevated CPK (228 Ui and CPK-MB (1748). Despite the use of inotropic agents and intensive supportive care, the cardiac function as monitored clinically and on echocardiography continued to deteriorate. ECG continued to show low voltage and no ST-T disturbances or conduction disturbances. The nadir blood counts were Hb 125 g/L. leukocytes 0. I x IO”/L, and platelets 41 X I0”iL. There war no bleeding diathesis or clinical evidence of infection. and all cultures were negative. The patient died on the 5th day after ABMT of cardiac failure. Permirsion was not granted for a postmortem examination. Cardiotoxicity is a serious adverse effect of antimalignancy cheniotherapeutic agents such as the anthracycline antibiotics 1 I and cyclophosphamide 121. Cardiac toxicity has been seen after combination therapy with
OAVlO SCHECHTER
A.J.J.T. REIN SHIMON SLAVIN Departments of Bone Marrow Transplantation and Cardiology, Hadassah University Hospital, Jerusalem, lsrael
REFERENCES I . Doroshow JH: Doxorubicin-induced cardiac toxicity. N Engl J Med 324:843-845, 1991. 2 . Goldberg M A . Antin JH. Guinan EC. Rappcport JM: Cyclophosphamide cardiotoxicity. an analysis of dosing as a risk Factor. Blood 6X:l 114-1 118. 1986. 3. Zulian G B . Selby P. Milan S. el al.: High dose melphalan. B C N U and eroposide with autologous bone inarrow transplantation for Hodgkin’s disease. Br J Cancer 59:631-635. 1989. 4. Chabner B A . Collinr JM, eds: “Cancer Chemotherapy: Principles and Practice.” Philadelphia: J.B. Lippincolt, 1990.
DermatomyositisAssociated With Acute Prornyelocytic Leukemia T o the Edimr: The association of dermatoniyositis (DM) with malignant neoplastic diseases is well known [I,2]. However. reports of DM preceding acute myelocytic leukemia are very rare. We describe a case of DM that was followed by acute promyelocytic leukemia (APL) and was moreover interestingly associated with seminoma. A 30-year-old man was admitted to our hospital because of myalgia. muscle weakness. and skin rash in April 1986. He was diagnosed as having DM by clinical signs, elevated serum muscle enzyme activity, electromyography. and skin biopsy. He was treated with high doses of prednisolone (I mgikg. daily). and improvement was seen in his clinical signs and laboratory data. However, an indurated testicular mass approximately 10 x 8 cm in size was subsequently noted. Echosonography demonstrated the image of a seminoma. He underwent orchidectomy. and histology of the testicular mass revealed seminoma. Moreover, he had a metastatic tumor infiltrating the lung, so he received four courses of combination chemotherapy (cisplatin, etoposide, peplomycin. and prednisolone). The lung mass subsequently disappeared, and his condition has remained good for 3 years.
Letters and Correspondence He presented to our institute wlth fever and general malaise in July 1990. Complete blood count showed 9 . 6 gidl, WBC 1.7 x 109/L, and platelets 85 X IO'iL. Bone marrow aspirate showed hypercellularity with 69.2% atypical promyelocytes with Auer rods. Cytochemical examination revealed that leukemic cells in the bone marrow were highly positive for peroxidase stain. Chromosomal analysis of marrow cells by G-banding revealed normal karyotype. lmrnunophenotyping of the leukemic cells was CD13 (54.8%) and CD33 (57.0%) positive. Thus, the patient was diagnosed as having APL. He was treated with behenoyl cytosine arabinoside, daunorubicin, 6-niercaptopurine, and prednisolone. A complete remission was achieved after I month, The patient remains in complete remission in June 1991. DM is a rare and severe disorder characterized by an inflammatory myopathy and typical cutaneous lesions [3]. Some reports have noted that patients with DM develop malignant neoplastic disease, and that the most common tumors associated with DM are those of the lungs. uterus, ovary, breast. prostate, and gastrointestinal tract 141. However, it is very unusual that DM was associated with APL and moreover with seminoma. T o our knowledge, this association has not been previously described. There are various possible causes for the APL seen in our patient. In this case, seminoma was treated with cytotoxic drugs. It is possible that APL occurred as a therapy-related leukemia. Therapy-related leukemias are known to occur as a side effect of treatment with cytotoxic drugs, particularly alkylating agents IS]. Moreover, therapy-related leukemias commonly accompany cytogenetic abnormality and inyelodysplastic changes [6,7]. However. in our patient the clinical presentation and evolution were not typical of therapy-related leukemias. In addition, alkylating agents were not used in this case. Thus, there was little possibility of therapy-related leukemia. Although an abnormal immune mechanism may have caused the APL, we cannot directly demonstrate it. We must also consider the possibility that the association of DM. APL. and seminoma in our patient may have been entirely coincidental. We conclude that awareness of this association may lead to earlier detection of malignancy. and hence to potentially more effective therapy.
243
Five Monoclonal Proteins in a Patient With Chronic Myelomonocytic Leukemia
To fhe Editor; Monoclonal gammopathies of undetermined significance (MGUS) constitute a heterogeneous group of diseases with a proliferation of a single clone of plasma cell homogeneous monoclonal proteins (M) without evidence of multiple myeloma, amyloidosis, rnacroglobulinemia. or other lymphoproliferative diseases [ I ] . These gammopathies can be associated with both hematologically benign and malignant diseases. as in other nonhematological processes. In approximately 2% of MGUS. biclonality can be seen 121. Isolated cases of triclonal antibodies have also been described in patients with plasma cell dyscrasia and acquired immunodeficiency syndrome (AIDS) and non-Hodgkin lymphomas 13.41. Nevertheless, as far as we know, no patient with five monoclonal proteins has even been described. This patient was diagnosed with chronic myelomonocytic leukemia on the basis of peripheral blood and bone marrow. The bone marrow karyotype shows a pathological metaphases of the 19 studied, one of them with trisomia of chromosome 8 and the other with a deletion of the short arm of chromosome 5. Blood and urine neuramidase are of 40 ngiml and 8 4 mgi24 hr respectively. Electrophoretic studies of total proteins produced the following results: total proteins 8.7 gidl albumin 4.2 gidl.. a , - G 0.5 gidl. a2-G 0.9 gidl. P-G I gidl (paraprotein p) y-G 2 . 3 gidl (paraprotein + y), and immunoglobulins IgG 1,730 nigidl, IgA 378 mgidl, and IgM 433 mgidl. High-resolution electrophoresis in agarose gel was carried out, which showed the existence of five proteins which could be identified through inmunoelectrophoresis and inmunofixation. With both techniques, two monoclonal bands corresponding to 1gG ( K and A). two monoclonal bands corresponding to IgA ( K + K), and a monoclonal band corresponding to IgM (A) were found. There was proteinuria in the urine of 15.9 mgi24 hr which was studied TOMOHIRO SUGAWARA by electrophoresis and inmunofixation. Two monoclonal bands were obKAZUYAW ENDO served IgG ( K and A) and one monoclonal band of IgA ( K ) as well as K and JUNKIMURA A free chains together with a heavy chain IgG (7). JUNNOMURA Neither chronic myelomonocytic leukemia nor monoclonal serum proKAZUMICHI FURUYAMAteins in urine proteins changed over 3 years of follow-up. This non-progresHIDEOHARIGAEsion of the disease IS an essential feature of benign monoclonal garnmopaKVOKO KANEOAthies by definition [Sl.
+
KAORU YOSHIN~GA
Second Department of lnternal Medicine, Tohoku University School of Medicine, Aobaku, Sendai, Japan
REFERENCES 1. Barnes BE: Derinatomysitis and malignancy: a review of the literature. Ann Intern Med 84:68-76. 1976.
2. Richardson JB, Callen JP: Dermatocyositis and malignancy. Med Clin North Am 73:1211-1220. 1989. 3 . Callcn JP: Dermatomyositis. Neurol Clin 5:379-403, 19x7. 4. Callen JP: Dermatomyositis: Current controversies 1987. Adv Dermatol 3:3-24. 1988. 5 . Greene MH. Boice JD. Greer BE, Blessing JA, Dembo AJ: Acute nonlymphocytic leukemia after therapy with alkylating agent5 for ovarian cancer-A study of five randomized clinical trials. N Engl J Med 307:1416-1421. 1982. 6. Michels SD, McKenna R W , Anher DC, Brunning RD: Therapy-related acute myeloid leukemia and myelodysplastic syndrome: A clinical and morphologic study of65 cases. Blood 65:136&1372. 1985. 7. Pedersen-Bjergdard J , Philip P: Cytogenetic characteristics of therapy-related acute nonlymphocytic leukaemia. preleukaeinia and acute myeloproliferative syndrome: correlation with clinical data for 61 consecutive cases. Br J Haematol
66:199-207. 1987.
A. VILLEGAS M. L ~ P ERueio Z M. ARROYO E. DEL POTRO Servicios de Hematologia y Bioquimica Hospital Universitario San Carlos Universidad Complutense de Madrid Madrid, Spain
REFERENCES I . Kyle RA: Monoclonal pammopathy of undetermined significance (MGUS): A review. Clin Haematol I1:123. 1982. 2 Kyle RA. Lust JA: Monoclonal gammopathies of undetermined significance.
Semin Hematol26:176. 1989. 3. Berg A R , Weissenburger DD. Linder J. Arnmitage JD: L)mphoplasmocytic lymphoma: Report of a case with three monoclonal proteins derived from a single neoplaatic clone. Cancer 57: 1794. 1986. 4. Ray RA, Schotters SB: Triclonal gammophathy in il patient with AIDS. Clin Chem 32:2Oc)O, 1986. 5 WaldeStriim JC: Benign monoclonal gammopathy. Acta Med Scaiid 216:435. 19x4.
