Abstract
Two boys with Down's syndrome, recognized at birth, developed acute myelogibrosis at the ages of 19 and 21 months. The disorder presented with anaemia and splenomegaly, and clinically resembled acute leukaemia, but bone marrow histology showed a bizarre pattern with generalized fibrosis, markedly increased reticulin, large reticulum cells, and giant cells resembling megakaryocytes. The children survived 6 and 11 months from diagnosis. A third case is quoted (Hillman and Forrester, 1968) which was also studied at this hospital; the features of all 3 cases are similar. There appears to be an increased incidence of acute myelofibrosis in children with Down's syndrome, which may be a further example of the instability of the haemopoietic system in the disease. In children with Down's syndrome and unusual leukaemia-like illness, histological examination of the bone marrow may be diagnostic.
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- Bernard J., Seligmann M., Loirat C., Chassigneux J., Basch A., Gueudet A. Splénomégalie myéloïde familiale. Nouv Rev Fr Hematol. 1967 Jul-Aug;7(4):499–506. [PubMed] [Google Scholar]
- DAMESHEK W. Some speculations on the myeloproliferative syndromes. Blood. 1951 Apr;6(4):372–375. [PubMed] [Google Scholar]
- DECASTELLO A. Osteomyelosklerose bei Vater und Tochter. Wien Klin Wochenschr. 1954 Sep 3;66(35-36):655–658. [PubMed] [Google Scholar]
- FUJINAMI T., SUGIYAMA T., TANKAWA H., UESUGI Y. [A case of megakaryocytic myelosis in a child]. Shonika Kiyo. 1961 May-Jun;7:226–234. [PubMed] [Google Scholar]
- Gorius J. B., Daniel M. T., Flandrin G., Denoel G. K. Fine structure and peroxidase activity of circulating micromegakaryoblasts and platelets in a case of acute myelofibrosis. Br J Haematol. 1973 Sep;25(3):331–339. doi: 10.1111/j.1365-2141.1973.tb01744.x. [DOI] [PubMed] [Google Scholar]
- Hayakawa H., Matsui I., Higurashi M., Kobayashi N. Hyperblastic response to dilute P.H.A. in Down's syndrome. Lancet. 1968 Jan 13;1(7533):95–96. doi: 10.1016/s0140-6736(68)90109-8. [DOI] [PubMed] [Google Scholar]
- Hillman F., Forrester R. M. Myelofibrosis simulating acute leukaemia in a female infant with Down's syndrome. Ir J Med Sci. 1968 Apr;7(4):167–173. doi: 10.1007/BF02946511. [DOI] [PubMed] [Google Scholar]
- Holden J. D., Garcia F. U., Samuels M., Dupin C., Stallworth B., Anderson E. Myelofibrosis with C monosomy of marrow elements in a child. Am J Clin Pathol. 1971 May;55(5):573–579. doi: 10.1093/ajcp/55.5.573. [DOI] [PubMed] [Google Scholar]
- KRIVIT W., GOOD R. A. Simultaneous occurrence of mongolism and leukemia; report of a nationwide survey. AMA J Dis Child. 1957 Sep;94(3):289–293. doi: 10.1001/archpedi.1957.04030040075012. [DOI] [PubMed] [Google Scholar]
- KUNDEL D. W., BRECHER G., BODEY G. P., BRITTIN G. M. RETICULIN FIBROSIS AND BONE INFARCTION IN ACUTE LEUKEMIA. IMPLICATIONS FOR PROGNOSIS. Blood. 1964 Apr;23:526–544. [PubMed] [Google Scholar]
- Krasilnikoff P. A. Myelofibrosis and myeloid leukaemia. Acta Paediatr Scand. 1967 Jul;56(4):424–429. doi: 10.1111/j.1651-2227.1967.tb15401.x. [DOI] [PubMed] [Google Scholar]
- LEWIS S. M., SZUR L. MALIGNANT MYELOSCLEROSIS. Br Med J. 1963 Aug 24;2(5355):472–477. doi: 10.1136/bmj.2.5355.468-a. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Miller J. M., Sherrill J. G., Hathaway W. E. Thrombocythemia in the myeloproliferative disorder of Down's syndrome. Pediatrics. 1967 Nov;40(5):847–850. [PubMed] [Google Scholar]
- Nagai K., Sugiyama Y., Isono Y., Yoshida K. An autopsy case of primary osteomyelosclerosis in a 6-year-old girl. Acta Pathol Jpn. 1969 Feb;19(1):81–93. doi: 10.1111/j.1440-1827.1969.tb00694.x. [DOI] [PubMed] [Google Scholar]
- Pochedly C., Ente G. Disseminated intravascular coagulation in a newborn with Down's syndrome. J Pediatr. 1968 Aug;73(2):298–298. doi: 10.1016/s0022-3476(68)80097-6. [DOI] [PubMed] [Google Scholar]
- ROSENBERG H. S., TAYLOR F. M. The myeloproliferative syndrome in children. J Pediatr. 1958 Apr;52(4):407–415. doi: 10.1016/s0022-3476(58)80061-x. [DOI] [PubMed] [Google Scholar]
- ROSS J. D., MOLONEY W. C., DESFORGES J. F. Ineffective regulation of granulopoiesis masquerading as congenital leukemia in a mongoloid child. J Pediatr. 1963 Jul;63:1–10. doi: 10.1016/s0022-3476(63)80296-6. [DOI] [PubMed] [Google Scholar]
- Sasaki M., Obara Y. Hypersensitivity of lymphocytes in Down's syndrome shown by mixed leukocyte culture experiments. Nature. 1969 May 10;222(5193):596–598. doi: 10.1038/222596b0. [DOI] [PubMed] [Google Scholar]
- Tobin M. S., Tan C., Argano S. A. Myelofibrosis in pediatric age group. N Y State J Med. 1969 Apr 15;69(8):1080–1083. [PubMed] [Google Scholar]
- Weinberger M. M., Oleinick A. Congenital marrow dysfunction in Down's syndrome. J Pediatr. 1970 Aug;77(2):273–279. doi: 10.1016/s0022-3476(70)80335-3. [DOI] [PubMed] [Google Scholar]