Myelofibrosis has been a confusing and controversial subject since Heuck's original
case in 1879. It is characterized by splenomegaly with active extramedullary
hematopoiesis, leukoerythroblastosis, poikilocytosis such as tear-drop cells and giant or
bizzare platelets in the peripheral blood, and fibrotic bone marrow.
Myelofibrosis is always a serious and a fatal disease, little influenced by treatment.
The etiology and pathogenesis are imperfectly understood, but it's relationship to other
myeloproliferative diseased is becoming better defined.
The purpose of this stuffy is to illustrate the clinical features and the quantitative
classification of the bone marrow fibrosis in 68 cases of myelofibrosis at the Severance
Hospital, Yonsei University College of Medicine from 1968 and 1977.
The results are as follows:
1. The agnogenic type was 11, and the secondary type 57. The associated diseases in
the secondary were chronic myelogenous leukemia (21 cartes), acute leukemia (17 cases),
metastatic carcinoma (8 cases), malignant lymphoma(5 cases), polycythemia vera (2
cases), multiple myeloma (2 cases), systemic lupus erythematosus (1 case), and
congenital syphilis (1 case) in order.
2. The mean age was 32.7±16.7 (2/1∼76) years [33.7±14.7 (14∼57) in the
agnogenic]. The M : F ratio was 1.3 : 1 (1 : 1.75 in the agnogenic).
3. The chief complaints were palpable mass and/or discomfort in the left upper
quadrant (26.5%), weakness (23.5%), fever (25.0%) and joint & back pain (13.2%) in
order.
The symptom duration of the agnogenic was 4.5±2.8 months, except 1 case of five
years' duration.
4. The physical findings revealed anemia (69.7%), fever (83.8%), hepatomegaly (59.1%),
splenomegaly (54.5%), and lymphadenopathy (30.0%) in order.
5. The hemoglobin below the normal was noted in 93.8%, reticulocytosis in 40.0%,
nucleated red cells in 63.1%, and tear-drop poikilocytosis in 96.7%.
6. The platelet count was decreased in 50.0% and increased in 19.4%. The giant
platelets were observed in 62.5%.
7. Leukopenia was noted in 29.7%, leukocytosis in 40.6%, immature forms in 48.4%,
and shift-to-left in 27.6%.
8. Dry tap wart noted in 83.6% (100% in the agnogenic). The megakaryocyte was
increased in 46.9% and decreased in 31.2%,
The degree of the fibrosis was "N" 9.3%, "+1" 15.4%, "+2" 33.8%, "+3" 21.5 %, and
"+4" 20.0%. There was no relationship between the degree of fibrosis of the marrow and
clinico-hematological findings.
9. Therapeutic measures in 60 cases were transfusion (37 cases), corticosteroids (27
cases), androgen preparations (7 cases), radiotherapy (5 cased) and folic acid (3 cases),
etc.
10. Excluding 13 cased in which terminal courses were unknown, 27 were improved,
15 not chanced, and 13 expired (6, 1, and 4, respectively, in the agnogenic). The causes
of death were sepsis in 4 (2 of the agnogenic), bleeding 3, and blastic crisis 3 (chronic
myelogenous leukemia).
11. The survival times in the 4 cases of the agnogenic whose death were confirmed,
were 3, 4, 8, and 19 months from their symptom onsets; and 23 drys, 1 day, 5 months
and 1 year, respectively, after their intial diagnosis.
As a conclusion, the agnogenic myelofibrosis in this country seems to develope in
younger age and to take more acute courses than in the western.
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