Blood, 1960, Vol. 15, No. 1, pp. 1-29.
© 1960 American Society of Hematology, Inc.
Refractory Anemia with Hyperplastic Bone Marrow
RICHARD W. VILTER 1,
THOMAS JARROLD 1,
JOHN J. WILL 1,
JOHN F. MUELLER 1,
BEN I. FRIEDMAN 1, and
VIRGINIA R. HAWKINS 1
1 Division of Hematology and Nutrition, Department of Internal Medicine, University of Cincinnati College of Medicine, the Cincinnati General Hospital, and the Cincinnati Veterans Administration Hospital, Cincinnati, Ohio.
1. The hematologic syndrome called refractory anemia with hyperplastic
bone marrow, aregenerative anemia, chronic bone marrow failure, pseudo-aplastic anemia, and many other terms, has been separated into five types
on the basis of clinical and hematologic studies on 23 patients carried out
over a period of 16 years. These groupings are probably highly artificial but
are made to facilitate presentation and description. As more is learned
of the chemistry of the bone marrow cells in patients with refractory anemia,
a more satisfactory classification will be possible.
2. Type 1 is characterized by bizarre chromatin in the normoblasts, mast
cell hyperplasia, hemosiderosis or hemochromatosis and a tendency for
spontaneous remissions to occur.
3. Type 2 is at first typified by complete or almost complete erythroid
aplasia. Hyperplasia of erythroid cells with maturation arrest and bizarre
chromatin pattern may occur later, and finally spontaneous remission may appear with considerable frequency. Mast cell hyperplasia, thymoma and
hemosiderosis have been noted.
4. Type 3, associated with exogenous toxins and the preleukemia state,
is characterized by pancytopenia and a bizarre chromatin pattern in the
normoblasts. Some of these patients may he classified as "DiGuglielmo
syndrome."
5. Type 4 also is typified by pancytopenia and hyperplasia of bone marrow but with cells of normal appearance. These patients responded partially
or completely when splenectomy was performed, and probably represent a
variant of the "hypersplenism" syndrome.
6. Type 5 is refractory megaloblastic anemia with typical cytologic changes
evident in all cell types similar to those found in pernicious anemia.
7. Hypotheses are proposed to explain these various types of anemia on
the basis of abnormalities in the metabolism of nucleic acids, particularly
DNA. Deficiencies of metabolites, inhibition of metabolic reactions by
exogenous or endogenous toxins, or by immune mechanism in which DNA
serves as haptene, are possible explanations.
Submitted on March 20, 1959
Accepted on June 29, 1959
