Kunihiko Yamauchi

Symptomatic intracranial haemorrhage in acute nonlymphoblastic leukaemia: analysis of CT and autopsy findings

Abstract We studied the CT and autopsy findings in patients with symptomatic intracranial haemorrhage (ICH) in acute nonlymphoblastic leukaemia (ANLL). From 1982 to 1994, 38 (20%) of 194 patients with ANLL were diagnosed as having ICH, by CT in 17 patients, by autopsy in 11 and by both examinations in 10. Intracerebral haemorrhage occurred in 30 patients. Twenty-four patients with subcortical haemorrhage were classified into three types: a single haematoma (7), clustered multifocal haematomas (11), and separated multifocal haematomas (6). Subarachnoid haemorrhage (SAH) occurred in 22 patients; 15 with subcortical haemorrhage, 1 with subdural haemorrhage (SDH) and 6 without any other ICH. SDH was also found in 4 patients with parenchymal haemorrhage or SAH or both. Concurrent, multiple haemorrhages consisting of various combinations of intracerebral haemorrhage, SAH and SDH are characteristic of ICH in ANLL. Multiple or confluent haematomas occur preferentially in subcortical brain.

Clinical significance of LEA-1 expression in adult acute myeloid leukemia

In this study, we examined expressions of several adhesion molecules (AdMs), i.e. leukocyte function antigen-1 (LFA-1: CD11a/CD18), Hermes homing receptor (CD44) and intercellular adhesion molecule-1 (ICAM-1: CD54), on leukemia cells from 51 adult patients with newly diagnosed acute myeloid leukemias (AMLs) to elucidate clinical significance of these AdM expressions. Those expressions in lymphoid malignancies have been correlated with tumor evolutions, but CD44 was detected in all the AML cases examined and CD54 expression did not associate with their clinical characteristics or outcomes. However, we found that LFA-1 expressions significantly correlated with splenomegaly, resistance to induction chemotherapies and short survival periods in AML patients.

Gallium-67 uptake in the salivary glands in chronic graft-versus-host disease after bone marrow transplantation

Ga-67 citrate scans were performed in a 17-year-old female patient after bone marrow transplantation for acute lymphoblastic leukemia. Ga-67 accumulated in salivary glands in which chronic graft-versus-host disease (GVH) was demonstrated pathologically. Ga-67 scan may be a sensitive and noninvasive test for detecting and monitoring the Sicca syndrome induced by chronic GVH.

PYRIDOXAL 5‐PHOSPHATE THERAPY IN A PATIENT WITH MYELODYSPLASTIC SYNDROME AND ADULT ONSET CONGENITAL ERYTHROPOIETIC PORPHYRIA

We report herein a patient who suffered from refractory anaemia (RA), a subtype of the myelodysplastic syndrome (MDS). with an acquired defect in porphyrin metabolism consistent with congenital erythropoietic prophyria (CEP). Marrow aspiration revealed a low percentage of ringed sideroblasts which did not fulfil the FAb criteria of refractory anaemia with ringed sideroblasts. However, we administered pyridoxal 5-phosphate (PLP). the active cofactor of pyridoxine (vitamin Bb). which is the established treatment for sideroblastic anaemia, to assess the effect on the haem biosynthetic pathway. The abnormality in the porphyrin metabolism associated with CEP improved with this treatment. This approach may be helpful as an alternative treatment to blood transfusion, charcoal or /I-carotene for CEP. In August 1989 a 63-year-old Japanese male noticed reddish brown urine and tingling pain, redness, swelling and thickening of sun-exposed skin. In September 1990 laboratory evaluations revealed normal serum aspartate aminotransferase, alanine aminotransferase and y-glutamyl transpeptidase, but elevated alkaline phosphatase concentrations. Neither the liver nor the spleen were palpable. His urine contained elevated concentrations of coproporphyrin (COP) (6735 pg/24 h) and uroporphyrin (3555 pg/24 h), but normal concentrations of 6-aminolevulinic acid and porphobilinogen. High-performance liquid chromatography revealed 96.3% of the urinary.UP0 to be the I isomer. Increased concentrations of porphyrins were also found in the erythrocytes. Fecal COP was markedly increased to 40.7 mg/d. Although the patient exhibited none of the usual signs of CEP in infancy and had no family history suggesting porphyria, these biochemical findings supported the diagnosis of CEP rather than porphyria cutanea tarda. Haematological data revealed pancytopenia with a haemoglobin concentration of 7.1 g/dl, a platelet count of 10 x 10y/l, a leucocyte count of 2.4 x 10y/l with 1% blasts, and 7 normoblasts per 100 leucocytes. Bone marrow analysis revealed hypercellularity with an increase in the erythroid series (76.6%). ringed sideroblasts (4% of the total erythroblast count), megaloblastoid features, but no maturation arrest in the myeloid series, Pelger-Huet-like anomalies in the neutrophils and haemosiderin-laden macrophages. Haemolysis was evident with a shortened erythrocyte life span (13.8 d; normal range 2 5-30 d), an elevated serum indirect bilirubin (1.0 mg/dl) and positive urinary haemoglobin. but no reactive reticulocytosis (2.45 x 10y/l). Plasma iron disappearance time was shortened to 37 min (normal half-life range 60-120 min). The percentage utilization of 59Fe for haemoglobin synthesis was only 18%. indicating ineffective erythropoiesis. Finally, a diagnosis of RA with adult onset CEP was made. Prednisolone therapy was initiated at a dose of 15 mg daily, but this did not reduce the excess excretion of urinary porphyrin and did not improve the progressive pancytopenia. Photosensitivity eruptions were totally unresponsive to oral pyridoxine. Instead, subcutaneous injection of pyridoxal 5-phosphate (PLP) was given at a dose of 30 mg/d. The urinary porphyrin concentration decreased to the normal values during the ensuing 5 months (Fig 1). The elevated serum indirect bilirubin concentration decreased to values within the normal range, and the urinary haemoglobin became negative within 2 months, however, the anaemia gradually progressed. The cutaneous lesions disappeared gradually over a period of 1 year. In March 1991 haematological findings showed a haemoglobin concentration of 3.9 g/dl, a leucocyte count of 6.9x1OY/1 and a platelet count of 0.2 x 10y/l. The reticulocyte count had decreased to 1.29 x 10y/l, and the erythroblasts had disappeared from the blood. Blasts began to circulate at less than 5% of the total leucocyte count. Bone marrow transformed to severe hypoplasia without an apparent increase in blasts 2.4%). Both Ham and sugar-water tests were negative. These results were consistent with hypoplastic MDS reported by Nand & Godwin (1988). Adult onset CEP has been described in only seven cases to date (Krameretal, 1965:Painetal. 1975; Westonetal, 1978: Deybach et al. 1981: Rank et al. 1990). Leucocytopenia and/ or thrombocytopenia was demonstrated in five of these seven

Spontaneous Remission with Cyclic Leukocytosis in Chronic Myelogenous Leukemia

A 41-year-old female with Philadelphia chromosome (Ph1)-positive chronic myelogenous leukemia was observed for 7 years. Cytotoxic therapy was not instituted until blastic crisis developed in the last 4 months. Successive hematologic measurements revealed cyclic fluctuations in blood leukocyte and platelet counts. These cycles had an average duration of 71 days during the 43 months observed. The oscillation amplitude decreased gradually over time, followed by hematologic remission until the last 4 months. The possible relationship between long-term survival and cyclic leukocytosis is discussed.

Pulmonary fibrosis with megakaryocytoid cell infiltration in accelerated phase of chronic myelogenous leukaemia

Summary. We report a patient in the accelerated phase of Philadelphia‐chromosome‐positive chronic myelogenous leukaemia who developed fibrosis in lungs, spleen and bone marrow. In the lungs, fibrosis was demonstrated in the alveolar septa which had been infiltrated by giant, bizarreshaped cells resembling the megakaryocytes in the bone marrow. The relationship between the fibrosis and megakaryocytoid cell infiltration in the lungs is discussed.

Myelodysplastic syndrome with thrombocytosis in a patient with Klinefelter's syndrome.

We report herein a patient with Klinefelters syndrome associated with refractory anemia with excess of blasts in transformation, a subtype of myelodysplastic syndrome (MDS). The MDS developed with karyotypic abnormality involving t(4;7)(q21;q11), and was characteristic of marked thrombocytosis and marrow infiltration by many atypical megakaryocytes. The patient also had diabetes mellitus and a disturbed immune system. To our knowledge, this is the fifth reported case of MDS in patients with Klinefelters syndrome in the literature.

Kinetics of indium-III-labeled eosinophils in two patients with eosinophilia.

The kinetics of IIIIn-labeled eosinophils was evaluated in two cases of cosinophilia by means of external and blood cell counting. In both cases, the labeled blood eosinophils decreased rapidly for a few h after injection and then increased progressively up to the 20th and the 12th h, respectively. The changes of radioactivity with time in the liver, spleen and lungs correlated with that in the blood eosinophils. However, in both cases radioactivity of the thoracic spine increased rapidly for a few h after injection of the labeled eosinophils and subsequently decreased. This result suggests that some of the eosinophils recirculate between the marginal pool of marrow and the intra vascular space.

Abnormalities of pulmonary blood flow during cold exposure in systemic lupus erythematosus.

The aim is to determine whether pulmonary blood flow is altered during cold exposure of the hands in individuals with systemic lupus erythematosus (SLE). Cold exposure was induced by immersion of the hands, one at a time, in 10°C water in nine SLE patients in whom pulmonary fibrosis had been ruled out by a routine examination. Lung perfusion was measured by continuous venous infusion of krypton 81 m.Cold exposure to at least one of the hands caused an 8 to 22% decrease of pulmonary blood flow in five of the nine patients. A 10 to 33% increase in flow developed in three of the nine patients during cold exposure of the hands. We conclude that these abnormalities in pulmonary blood flow during cold exposure were revealed in SLE patients with scarcely any clinical symptoms.

Identification of circulating micromegakaryocytes in a case of erythroleukemia

A 26‐year‐old man with erythroleukemia was found to have circulating micromegakaryocytes. Megakaryocytic features were defined by morphologic and cytochemical studies using light and electron microscopy with platelet‐megakaryocyte peroxidase staining. This appears to be the first reported instance of erythroleukemia with circulating micromegakaryocytes.