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| Acute mast cell leukemia Classification and external resources |
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| ICD-10 | C94.3 |
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| ICD-9 | 207.8 |
| ICD-O: | M9742/3 |
| DiseasesDB | 30102 |
| MeSH | D007946 |
Mast cell leukemia is an extremely aggressive subtype of acute myeloid leukemia that usually occurs de novo but can, rarely, evolve from transformation of chronic myeloid leukemia into the more aggressive acute myeloid leukemia. In a small proportion of cases, acute mast cell leukemia may evolve from a more progressive form of systemic mastocytosis. The diagnosis of acute mast cell leukemia by the WHO criteria includes the requirement for a prevalence of 20% neoplastic mast cells in marrow and 10% in blood. [1] If the mast cells represent less than 10% of blood cells, the tumor is called "aleukemic" mast cell leukemia.
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Cytochemistry
Cytochemical properties of the leukemic cells must be typical of mast cell derivation (presence of metachromatic granules staining with alpha-naphthyl chloroacetate esterase, but not with peroxidase). [2] Mast cell tryptase is an enzyme contained in mast cell granules. Mast cell numbers are best estimated by tryptase immunostaining because very poorly granulated cells may stain very weakly if at all for alpha-naphthol chloroacetate esterase. [3]
Tumor Markers
The leukemic cells usually are strongly positive for CD13, CD33, CD68, and CD117. Characteristically, basophil (e.g. CD11b, CD25, CD123) and monocyte markers (CD14, CD15) are absent. [4] Malignant mast cells overexpress the anti-apoptosis gene, bcl-2. [5]
Clinical course
Acute mast cell leukemia is a rapidly progressive disorder with leukemic mast cells in blood and in large numbers in marrow. The common signs and symptoms include fever, headache, flushing of face and trunk. [6] [7] The typical cutaneous mast cell infiltrates of urticaria pigmentosa are usually not present before, during, or after diagnosis in patients who have mast cell leukemia. [8]Symptoms include abdominal pain, bone pain, and peptic ulcer which are more prevalent than in other subtypes of acute myeloid leukemia. These former symptoms are due to relase of a substance called histamine from neoplastic mast cells. [9] Enlargement of the liver and spleen, or hepatosplenomegaly is characteristic. [10] The mast cells release also many anticoagulants like heparin which can lead to serious bleeding. Liver and splenic dysfunction also contributes to hemorrhage. [11]
Laboratory Findings
Total serum tryptase is elevated in mast cell leukemia. Normal total(alpha + beta) serum tryptase is approximately 6 micro g/L (range 0 to 11 micro g/L). Values of several hundred micro g/L are characteristic of mast cell leukemia. [12] Plasma and urinary histamine levels are frequently elevated in mast cell leukemia. Histidine decarboxylase (HDC) is the enzyme that catalyzes the reaction which produces histamine from histidine. Measurement of histidine carboxylase in the marrow cells of patients with mast cell leukemia is a very sensitive marker of mast cells. [13]
Therapy
Immunoglobulin E (IgE) is important in mast cell function. Immunotherapy with anti-IgE immunoglobulin raised in sheep resulted in a transient decrease in the numbers of circulating mast cells in one patient with mast cell leukemia.[14] Although splenectomy has led to brief responses in patients with mast cell leukemia [15] [16] no firm conclusions as to the efficacy of this treatment are possible. Chemotherapy with combination of cytosine arabinoside and either idarubicin, daunomycin, or mitoxantrone as for acute myeloid leukemia should be considered.
Prognosis
Acute mast cell leukemia is extremely aggressive and has a grave prognosis. In most cases, multiorgan failure including bone marrow failure develops over weeks to months. [17] Medial survival after diagnosis is only about 6 months. [18]
References
- ^ Lichtman MA and Segel GB, "Uncommon phenotypes of acute myelogenous leukemia: basophilic, mast cell, eosinophilic, and myeloid dendritic cell subtypes: a review."; Blood Cells Mol Dis. 2005 Nov-Dec;35(3):370-83.
- ^ Kufe D et al: Holland Frei Cancer Medicine, 5th ed. BC Decker, 2000.
- ^ Lichtman MA and Segel GB, "Uncommon phenotypes of acute myelogenous leukemia: basophilic, mast cell, eosinophilic, and myeloid dendritic cell subtypes: a review."; Blood Cells Mol Dis. 2005 Nov-Dec;35(3):370-83.
- ^ P. Valent, 1995 Mack-Forster Award Lecture. Review. Mast cell differentiation antigens: expression in normal and malignant cells and use for diagnostic purposes, Eur. J. Clin. Invest. 25 (1995) 715– 720
- ^ Cervero C, Ezcribano L, San Miguel JF, et al. Expression of bcl-2 by human bonebmarrow mast cells and its overexpression in mast cell leukemia. Am J Hematol 1999;60:191.
- ^ M.T. Daniel, G. Flandrin, J. Bernard, Acute mast-cell leukemia. Cytochemical and ultrastructural study, about a particular case, Nouv. Rev. Fr. Hematol. 15 (1975) 319– 332
- ^ M.T. Le Cam, P. Wolkenstein, A. Cosnes, H. Bocquet, P. Gaulard, M. Tulliez, et al., Acute mast cell leukemia disclosed by vasomotor flushing, Ann. Dermatol. Venereol. 124 (1997) 621– 622.
- ^ Travis WD, Li CY, Hoagland HC, et al. Mast cell leukemia: report of a case and review of the literature. Mayo Clin Proc 1986;61:957.
- ^ P. Valent, W.R. Sperr, P. Samorapoompichit, et al., Myelomastocytic overlap syndromes: biology, criteria, and relationship to mastocytosis, Leuk. Res. 25 (2001) 595– 602.
- ^ Kufe D et al: Holland Frei Cancer Medicine, 5th ed. BC Decker, 2000.
- ^ Cather J. C., and Menter M. A., MD, "Red-brown skin lesions and pruritus", BUMC Proceedings 2000;13:179-199.
- ^ W.R. Sperr, J.H. Jordan, M. Fiegl, L. Escribano, C. Bellas, S. Dirnhofer, et al., Serum tryptase levels in patients with mastocytosis: correlation with mast cell burden and implication for defining the category of disease, Int. Arch. Allergy Immunol. 128 (2002) 136– 141.
- ^ M.T. Krauth, H. Agis, K.J. Aichberger, I. Simonitsch, L. Muellauer, M. Mayerhofer, H.P. Horny, P. Valent, Histidine carboxylase (HDC) as novel marker of immature neoplastic mast cells in systemic mastocytosis, Blood 104 (11) (2004) 272b (Part 2).
- ^ I. Ribeiro, I.R. Carvalho, M. Fontes, F. Lima, R. Matos, B.A. Anderson, L.S. Uva, Eosinophilic leukaemia with trisomy 8 and double gammopathy, J. Clin. Pathol. 46 (7) (1993 Jul.) 672–673.
- ^ Travis WD, Li CY, Hoagland HC, et al. Mast cell leukemia: report of a case and review of the literature. Mayo Clin Proc 1986;61:957.
- ^ Dalton R, Chan L, Bitten E, Eridani S. Mast cell leukemia: evidence for bone marrow origin of the pathological clone. Br J Haematol 1986;64:397.
- ^ Hoffman R, Benz E, Shattil S, Furie B, Cohen H, "Hematology: Basic Principles and Practice", Churchill Livingstone, 4th ed, 2004.
- ^ Travis WD, Li CY, Hoagland HC, et al. Mast cell leukemia: report of a case and review of the literature. Mayo Clin Proc 1986;61:957.
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