Case 1. The patient, a fifty-four year old man, was admitted to the Niigata University Hospital because of anemia and gingival bleeding in november 1970. Physical examination on admission showed slight enlargement of the liver. White blood cell count was 25, 200 with 54% of monocytoid cells. The bone marrow puncture revealed marked proliferation of monocytoid cells (71.4%). PAS reaction of the erythroblasts was negative. The nucleated erythroblasts were seen in the peripheral blood at the rate of 10 per 200 WBC during the course. The treatment was ineffective and the patient died because of gastrointestinal bleeding on November 16, 1971. Both clinical and pathological diagnoses were myelo-monocytic leukemia (Naegeli type).
Case 2. The patient, a 29 year old woman, had an onset of anemia and pneumonia in December 1969. She was admitted to the Niigata University Hospital in February 1970. Physical examination on admission showed anemia and hepatosplenomegaly. White blood cell count was 44, 000 with 85% of monocytoid cells. The bone marrow puncture showed marked proliferation of monocytoid cells (73.2%). PAS reaction of the erythroblasts was positive. The marked increase of the erythroblasts was seen in the peripheral blood at the rate of 195per 200 WBC during the course. The patient died because of bacterial infection in August 1971. Clinical diagnosis was erythro-leukemia (di Guglielmo syndrome) but the autopsy could not be performed.
It is presumed that proliferation of the erythroid series, associated with absolute monocytosis, might be one of the causes of the relatively chronic course.

View full abstract

The haematologic abnormalities were examined in forty autopsied cases with complicated cardiovascular anomalies in our hospital.
Five cases of Asplenia Syndrome and other thirty five cases of CHDs with normal spleen were included in this study. These haematologic examinations includes the appearance of Howell-Jolly Bodies in peripheral red blood cells, new inclusion bodies (postsplenectomy vacuoles) in peripheral red blood cells detected with phase contrast microscope in 40 cases, and pocked erythrocytes in peripheral red blood cells with interference contrast microscope in 25 cases.
The results of this study showing the clearcut difference between Asplenia Syndrome and other severe CHDs with normal spleen were as follows;
1) In five cases of Asplenia Syndrome, the postsplenectomy vacuoles in peripheral red blood cells were observed in all five cases, with high appearance ratio (10-40% of the observed number of red blood cells), and with characteristic large (0.8-1.1μ in diameter), round, punched-out shape in morphologic features.
2) The other hand, in the other 35 cases with severe CHDs with normal spleen, nonspecific small (0.1-0.4μ in diameter), round shaped vacuoles were obsered in only 15% (5 out of 35 cases) of cases, and also with low appearance ratio (0.2-0.5%).
3) In all cases of Asplenia Syndrome, the pocked erythrocytes were observed with high appearance ratio (28-38% of red blood cells), and with characteristic morphologic features in large, creater-like shape (0.8-1.1μ in diameter).
4) The other hand, in the other 20 cases of severe CHDs, the small, non-characteristic pocked erythrocytes were observed with low appearance ratio (0.1-0.2%) in only 25% (5 out of 20 cases).
In addition to the appearance of Howell-Jolly Bodies in peripheral red blood cells, these results of the morphologic features of postsplenectomy vacuoles and pocked erythrocytes in periphera lred blood cells were thought to be clinically useful and pathognomonic for the antemortem diagnosis of Asplenia Syndrome.

View full abstract

The haematologic abnormalities were examined in 210 cases in our hospital. Eight cases of clinical Asplenia Syndrome with characteristic cardiovascular malformations, 10 cases of adults with splenectomy and control groups (10 premature babies, 10 neonates, 20 infants and 152 the other CHDs) were included in this study. In these haematologic examinations included, the appearance of Howell-Jolly Bodies in peripheral red blood cells, detected with microscope, Postsplenectomy Vacuoles in peripheral red blood cells with phase-contrast microscope and Pocked Erythrocytes in peripheral red blood cells with interference-contrast microscope.
The results of this study showed the same pathognomonic findings in all cases of clinical Asplenia Syndrome and of adults with splenectomy as follow;
1) Howell-Jolly Bodies with appearance ratio (0.9-6.2%, 0.4-4.1% in both groups.)
2) Characteristically large (0.8-1.1μ in diameter), round, punched-out shaped postsplenectomy vacuoles in peripheral red blood cells with PCM, were observed in both groups, with high appearance ratio (10-85%, 10-60% in both groups, out of the observed number of red blood cells).
3) Pocked erythrocytes with characteristically large (0.8-1.1μ in diameter), deep creater, were observed with ICM, with high appearance ratio (10-75%, 10-65% in both groups).
The other hand, in the control groups, no Howell-Jolly Bodies and non-specific, small (0.4μ or smaller in diameter), round shaped postsplenectomy vacuoles, with low appearance ratio (0.1-2.0%, 0.3-2.0%, 0.1-1.0% and 0.1-2.0% in each group), were observed in 70%, 90%, 30% and 20% out of examined cases in each group, and pocked erythrocytes with non-specific, small (0.4μ or smaller diameter), shallow creater with low appearance ratio (0.1-0.9%, 0.3-2.0%, 0.1-0.9% and 0.1-2.0%), were observed in the same cases, in whom postsplenectomy vacuoles were observed.
In addition to the appearance of Howell-Jolly Bodies, these results of the morphologic features of postsplenectomy vacuoles and pocked erythrocytes were thought to be usefull and much pathognomonic for the antemortem diagnosis of Asplenia Syndrome. The criteria for the antemortem diagnosis of Asplenia Syndrome was designed in our hospital as follow;
The positive findings of generalized marked cyanosis, visceral heterotaxy, symmetrical liver, persistent bilateral SVCs and cavo-aortic juxtaposition, are much suggestive of Asplenia Syndrome.
In addition, the clinical diagnosis of Asplenia Syndrome will be established with assurance by the positive findings as follow;
1) The appearance of Howell-Jolly Bodies.
2) The appearance of postsplenectomy vacuoles with PCM, with high appearance ratio (20-85%) and with morphologically characteristic features of large (0.8-1.1μ in diameter), round, punched-out shape.
3) The appearance of pocked erythrocytes with ICM, with high appearance ratio (10-60%), with morphologically characteristic features of large (0.8-1.1μ in diameter), deep creater.
4) The absence of the splenic artery (celiacography after ventriculography or aortography).

View full abstract

Patient was of 40 year-old male with fever, severe muscular pain and polyarthralgia.
He had been suffered from these symptoms scince June 1975, and was admitted to Akashi Municipal Hospital on July 15. The arteriolar inflammation peculiar to periarteritis nodosa (PN) was observed with his biopsied specimen of leg nodule soon after admission, and he was diagnosed as PN.
40mg/day of prednisolone was administrated to him for 3 weeks immediatory after diagnosis without response. Therefore 100mg/day of azathioprine with 40mg/day of prednisolone was administrated, but had no effect for further 5 weeks. Then 1, 500mg/day of t-AMCHA was orally given to the pt. After few days, the pain and inflammatory symptoms were obviously improved.
Antiplasmic compound “t-AMCHA” has also anti-inflammatory effect, and this is generally different from antiplasmic effect. The leading report of the pharmacological mechanism of this anti-inflammatory effect is said to be, in a part, inactivation of ACTH, but Yamasaki has reported that anti-inflammatory effect of t-AMCHA was observed in adrenolectomized rat. Therefore, clear pharmacological mechanism of t-AMCHA is as yet obscure.
Judging from immediate anti-inflammatory effect of t-AMCHA in our case, we speculate the anti-inflammatory effect is not brought through adrenal gland. And we think t-AMCHA is one of the adequate agent in the treatment of PN, especilly in a non-responsive case to glucocorticoid and/or immunosuppressive therapy.

View full abstract