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Year : 2008  |  Volume : 51  |  Issue : 1  |  Page : 118-120
Simultaneous appearance of dual malignancies of hematopoietic system-multiple myeloma and acute myeloid leukemia

1 Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, UP, India
2 Department of Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, UP, India

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We herein report a case of denovo and simultaneous appearance of multiple myeloma and acute myeloid leukemia in a 58-year-old female patient, without prior exposure to chemotherapy or radiotherapy. This case is reported because of its extreme rarity.

Keywords: Acute myeloid leukemia, dual malignancies, multiple myeloma

How to cite this article:
Shukla J, Patne SC, Singh N K, Usha. Simultaneous appearance of dual malignancies of hematopoietic system-multiple myeloma and acute myeloid leukemia. Indian J Pathol Microbiol 2008;51:118-20

How to cite this URL:
Shukla J, Patne SC, Singh N K, Usha. Simultaneous appearance of dual malignancies of hematopoietic system-multiple myeloma and acute myeloid leukemia. Indian J Pathol Microbiol [serial online] 2008 [cited 2022 May 16];51:118-20. Available from: https://www.ijpmonline.org/text.asp?2008/51/1/118/40422

   Introduction Top

The development of acute myeloid leukemia (AML) in patients receiving chemotherapy in the form of alkylating agents for the treatment of multiple myeloma is well described in the literature. However, the simultaneous occurrence of the dual malignancies of hematopoietic system without prior exposure to any chemotherapy or radiotherapy is extremely rare with the appearance of only sporadic case reports in the literature. [1],[6],[8] After an extensive search, we could find not more than 20 such cases that are reviewed and compared [Table - 1]. Herein, we are reporting such a case of multiple myeloma that coexisted with AML in a 58-year-old female who was not exposed to any chemotherapy or radiotherapy prior to presentation.

   Case History Top

A 58-year-old female presented with the spontaneous fracture of the right arm, pain in chest and back, paleness of the body and epistaxis for last 2 months. On general examination, she was severely pale with a temperature of 101°F; bilateral pitting pedal edema and bony tenderness were present. Systemic examination revealed hepatomegaly (3 cm) and splenomegaly (4 cm) without lymphadenopathy. No other significant physical findings were present on detailed systemic examination.

Pathological findings

Laboratory investigations revealed the following: hemoglobin - 42g/L, total leucocyte count - 4.3 x 10 9 /L and platelet count - 25 x 10 9 /L. Differential white cell count showed blasts 4%, promyelocytes 2%, myelocytes and metamyelocytes 10% and lymphocytes 84%. One blast cell showed Auer rod in the cytoplasm [Figure - 1]. Red cells were normocytic, normochromic and showed rouleaux formation. Nucleated red cells were 1/100 WBCs. Platelets were markedly reduced. Bone marrow aspirate was hypercellular, mainly showing two populations of cells - myeloid and plasma cells in a ratio of 1.5:1. These two populations of cells varied considerably in proportion in different areas of bone marrow [Figure - 2]. Amongst myeloid cell population, 68% cells were blast ells; 12%, promyelocytes; and others were myelocytes and metamyelocytes. The blast cell had myeloid morphology and stained positive with Sudan Black B. Plasma cells were 40% amongst all nucleated cells, and many of them showed abnormal morphology in the form of bi- and tri-nucleation. Erythropoiesis was markedly depressed with the presence of only few megaloblastic erythroid cells. Occasional megakaryocytes were observed, which were morphologically normal.

In view of extreme plasmacytosis of the bone marrow, serum and urine, electrophoresis was carried out which showed monoclonal band (M peak) in γ region that was identified as IgG 'λ by immunoelectrophoresis. The IgG level was 64g/L; only traces of IgM and IgA were evident. Bence-Jones protein in the urine was also positive. Skull and chest X-ray showed multiple lytic lesions. Amongst other investigations: total serum protein was 72 g/L; serum calcium, 2.3 mmol/L; serum phosphorus, 2.1 mmol/L; blood urea nitrogen, 30.16 mmol/L; serum creatinine, 114.3 µmol/L; and alkaline phosphatase, 880 U/L. USG abdomen showed the hydronephrosis of the left kidney.

Based on the above findings, the case was diagnosed as multiple myeloma co-existent with acute myeloid leukemia (AML-M2). The treatment of such cases is not well defined. Therefore keeping in view of the aggressive nature of the AML, treatment by anthracyclins and cytosine was instituted, but the patient was lost within a month owing to multiple complications including septicemia and renal failure.

   Discussion Top

The co-occurrence of multiple myeloma with AML at presentation is extremely rare. Therefore, whenever plasmacytosis with AML exists, reactive plasmacytosis must first be ruled out by carrying out investigations of multiple myeloma (monoclonal gammopathy) as reactive plasmacytosis may occur in 5-10% cases of AML. [9] Next, we have to make sure that either of them is not therapy related. The present case fulfills the criteria for the twin diagnosis of multiple myeloma and AML. It also offers further evidence that previous chemotherapy is not necessary for the development of acute leukemia in myeloma patients. Several pathophysiologic mechanisms have been proposed for explanation. These include a disorder of multipotent stem cells, exposure to common environmental risk factors and repeated infections. [6],[8] We feel that multiple myeloma being a slowly developing disease may have been subclinically harbored in this patient from many years. The decreased immune surveillance efficiency secondary to the myeloma may have resulted in a failure to eliminate incipient clones of leukemic cells. In this circumstance, multiple myeloma would precede and predispose to the development of leukemia.

As the number of patients with simultaneous appearance of both multiple myeloma and AML are very small, there is no established treatment of choice. The prognosis of such patients remains poor and no case of remission has ever been reported. Allogenic stem cell transplantation probably remains the best option where feasible. Recently, an experimental study showed that two drugs - tipifarnib and bortezomib are synergistic and overcome cell-adhesion mediated drug resistance in the microenvironment models of multiple myeloma and AML. [10] This may provide the preclinical rationale for the trial testing of these two drugs combinations in patients with simultaneous appearance of multiple myeloma and AML.

   References Top

1.Parapia L, Abbott CR, Masters G, Roberts BE. Simultaneous presentation of acute myelomonocytic leukaemia and multiple myeloma. Acta Haematol 1982;68:153-6.  Back to cited text no. 1  [PUBMED]  
2.Annino L, Martino P, Barsotti P, Serra P, Marinozzi V, Mandelli F. Multiple myeloma and acute myelomonocytic leukemia: Simultaneous occurrence without previous chemotherapy. Acta Haematol 1980;64:195-200.  Back to cited text no. 2  [PUBMED]  
3.Naparstek E, Leiserowitz R, Gamliel H, Polliack A. Simultaneous presentation of plasma cell and monocytic leukemia with a subacute clinical course. Acta Haematol 1982;68:249-55.  Back to cited text no. 3  [PUBMED]  
4.Raz I, Polliack A. Coexistence of myelomonocytic leukaemia and monoclonal gammopathy or myeloma: Simultaneous presentation in three patients. Cancer 1984;53:83-5.  Back to cited text no. 4  [PUBMED]  
5.Dash S, Sarode R, Day P, Sehgal S. An association of acute myeloid leukaemia and multiple myeloma: A case study. Indian J Cancer 1991;28:45-7.  Back to cited text no. 5    
6.Luca DC, Almanaseer IY. Simultaneous presentation of multiple myeloma and acute monocytic leukemia. Arch Pathol Lab Med 2003;127:1506-8.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Yanagimoto M, Usuki K, Iijima K, Hirai Y, Iki S, Urabe A. Simultaneous occurrence of acute myeloid leukemia with multilineage dysplasia and multiple myeloma. Rinsho Ketsueki 2003;44:19-24.  Back to cited text no. 7  [PUBMED]  
8.Attili S, Lakshmiah KC, Madhumati M. Simultaneous occurrence of multiple myeloma and acute myeloid leukaemia. Turk J Haematol 2006;23:209-11.  Back to cited text no. 8    
9.Wulf GG, Jahns-Streubel G, Hemmerlein B, Bonnekessen K, W φrmann B, Hiddemann W. Plasmacytosis in acute myeloid leukemia: Two cases of plasmacytosis and increased IL-6 production in the AML blast cells. Ann Hematol 1998;76:273-7.  Back to cited text no. 9    
10.Yanamandra N, Colaco NM, Parquet NA, Buzzeo RW, Boulware D, Wright G, et al . Tipifarnib and bortezomib are synergistic and overcome cell-adhesion mediated drug resistance in multiple myeloma and acute myeloid leukemia. Clin Cancer Res 2006;12:591-9.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]

Correspondence Address:
Jyoti Shukla
Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, UP
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.40422

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  [Figure - 1], [Figure - 2]

  [Table - 1]

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