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We performed an HLA-mismatched T cell non-depleted bone marrow transplant on a 53-year-old man with acute erythroleukemia that was highly resistant to conventional remission-induction chemotherapy. After conditioning that included total body irradiation, the patient received a two-HLA-antigen-mismatched bone marrow graft harvested from his sister using tacrolimus and methotrexate for graft-versus-host disease (GVHD) prophylaxis. He successfully established rapid engraftment accompanied by steroid-responsive GVHD localized to the skin. Although bone marrow samples on day 31 and day 66 disclosed a complete remission with full donor chimerism, the patient relapsed and died of pulmonary infection on day 154. There is evidence that tacrolimus is effective in alleviating GVHD. Selected patients who have partially mismatched related donors with less HLA disparity may benefit from tacrolimus-based T cell non-depleted bone marrow transplants because of the more potent graft-versus-leukemia effect that can be expected compared to transplants using T cell depleted inoculum.

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A 38-year-old woman with acute monoblastic leukemia developed severe continuous pain in the left arm while she was in hematologic remission following both systemic and intrathecal chemotherapy. A nerve conduction study (NCS) showed marked decrease of amplitude in the left ulnar nerve, consistent with infiltration of leukemic cells. The pain in the arm was reduced by irradiation to the left brachial plexus, but right facial nerve palsy occurred. No improvement was achieved by systemic and intrathecal chemotherapy plus irradiation to the whole brain and right parotid. After sometime, she complained of pains in the legs and right foot drop. NCS showed amplitude decrease in bilateral peroneal nerve. Throughout the course, bone marrow remained in complete remission, and no signs of meningeal leukemia were obtained. A treatment with high dose Ara-C appeared to be effective for the pain in the legs. The foot drop, however, persisted and peripheral neuropathy progressed even after high dose Ara-C therapy. Peripheral nerve involvement in acute leukemia appears to be rare, and even more so in case of hematologic remission. The blood-nerve barrier may allow some malignant cells to escape from cytotoxic agents. Therefore, irradiation or high dose Ara-C therapy would seem to be rational approaches to the problem.

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Adult T-cell leukaemia (ATL) is a unique type of T-cell malignancy closely associated with human T-cell leukaemia virus-1 (HTLV-1). Despite frequent descriptions of hypercalcaemia, cases accompanied by diabetes insipidus or syndrome of inappropriate secretion of anti-diuretic hormone (SIADH) in ATL patients have rarely been reported. We present an unusual case of ATL with various abnormalities in his endocrine and metabolic systems involving anterior pituitary function, thyroid function, lipid metabolism and Ca metabolism. Some of these abnormalities were considered to arise from infiltration or leukaemic cells into systemic organs after elimination of the above symptoms. Clinical and haematological data showing improvement following chemotherapy are also presented.

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We have determined a single amino acid substitution in a new phosphoglycerate kinase (PGK) variant, PGK Shizuoka, associated with chronic hemolysis and myoglobinuria. PGK Shizuoka had an extremely low enzyme activity with normal kinetic properties and normal electrophoretic mobility. Total blood cell RNA of the patient was reverse-transcribed and amplified by the polymerase chain reaction. A single nucleotide substitution from guanine to thymine at position 473 of PGK messenger RNA was found. This nucleotide change causes a single amino acid substitution from Gly to Val at the 157th position, which is located in the NH2-terminal domain of the enzyme. This mutation creates a new Bst XI cleavage site in exon 5, and we thus confirmed the mutation in the variant gene. The replacement of Gly by Val is considered to affect enzyme catalysis.

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A case of ET associated with angina pectoris is presented. Angiography showed a 3.0-cm long mosaic-like thrombus shadow consisting of small filling defects in the proximal left anterior descending artery. The lesion could not be reduced with warfarin, ticlopidine, trapidil, urokinase or melphalan. Coronary artery bypass grafting was performed successfully.

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An 80-year-old male was admitted because of dizziness and palpitation. Laboratory investigation revealed pancytopenia. A bone marrow aspirate showed a markedly hypocellular marrow with 41.6% blast cells. Peroxidase activity was negative and PAS reaction was block positive in the blast cells. Surface markers of these cells were positive for HLA-DR antigen and partially positive for CD13 (MY7). Other markers, such as T, B or myeloid antigens were all negative. These blast cells were classified as L1 according to the FAB system but suggested essentially unclassifiable in cell differentiation. The patient was treated successfully with vincristine and prednisolone and induced into complete remission although repeated marrow examination findings revealed hypocellular. As for the classification of hypoplastic leukemia, lymphoid or primitive "stem cell" leukemia also should be considered as other categories of acute leukemias and be treated according to each case.

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A 71-year-old woman with neutrocytopenia (690/mm3) was treated with prednisolone before coronary surgery. After the normalized hemogram with increased leukocyte counts, aorto-coronary bypass grafting with saphenous vein was performed for left main trunk disease. Her convalescence was quite stable without any infectious or adrenal problems. It was meaningful to treat neutrocytopenia with steroids in order to predict the hematological reaction to stress before the operation.

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Two patients with refractory anemia with excess of blasts in transformation having entered remission and in continuing complete remission with the small-dose cytarabine therapy (SDCA) are reported. SDCA was the only chemotherapy used in these two patients. Cytarabine was injected subcutaneously every 12 hours at doses 15 mg and 10 mg, respectively, in cases 1 and 2. Case 1, a 60-year-old woman, achieved remission after a 14-day course of SDCA. Thereafter, she received SDCA for seven consecutive days every month or every other months in the first year and every two or three months in subsequent years. Her remission has been maintained for 57 months. Case 2, a 76-year-old man, attained a remission after three courses of SDCA, each consisting of 16, 11 and 14 days, respectively. SDCA was then repeated every month for 6 consecutive days. He is still in remission after 33 months. Whereas SDCA caused severe bone marrow suppression during induction, it was well tolerated when given in remission without necessitating any supportive measure.

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A 52 year-old woman with systemic amyloidosis complicated with multiple myeloma died suddenly of intraperitoneal hemorrhage due to spontaneous rupture of the spleen and liver. Autopsy revealed multiple myeloma involving the bone marrow and diffuse amyloidosis involving the liver, spleen, kidneys, heart, bone marrow, lymph nodes, lungs, gastrointestinal tract, thyroid, skin and adrenal glands. The splenic red pulp and the hepatic parenchyma were replaced by masses of amyloid. Amyloid deposits were also numerous in the walls of blood vessels and linearly in the intracapsular regions of both the liver and spleen. This is the eighth case of spontaneous rupture of the spleen and the second case of spontaneous rupture of the liver in association with systemic amyloidosis.

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A case of generalized amyloidosis associated with cyclic neutropenia is presented. A 24-yr-old female with cyclic neutropenia died from intestinal obstruction caused by necrosis and perforation of the small intestine. Post-mortem examination revealed generalized amyloidosis involving almost all organs. Amyloid deposits were prominent, especially in the alimentary tract, kidneys, spleen, and small blood vessels. As has been suggested in gray collie dogs with congenital cyclic neutropenia known to develop secondary amyloidosis in adulthood, an increase of antigenic stimulation during the intermittent bouts of acute infections may be one of the factors responsible for the development of secondary amyloidosis in this case. Although the association of amyloidosis and cyclic neutropenia in man has rarely been described, it is probable that amyloidosis is not a rare complication of human cyclic neutropenia, considering that patients with this hematologic disorder are chronically exposed to excessive antigenic stimulation.

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An autopsy case of angio-immunoblastic lymphadenopathy with dysproteinemia (Frizzera et al. 1917) or immunoblastic lymphadenopathy (Lukes and Tindle 1975) is reported. Clinical pictures and morphologic characteristics of affected organs were typical of this disease. In spite of combination chemotherapy, the patient took a rapid fatal course. Post-mortem examinations disclosed involvement of the lymph nodes, liver, lungs, kidneys and skin. Cellular infiltrates in the kidney were more monomorphous, suggesting the potential for the development of immunoblastic sarcoma. Electron microscopies of the affected lymph nodes revealed the proliferation of immunoblasts characterized by moderate amount of clear cytoplasm with abundant polyribosomes and by large nuclei with prominent nucleoli. Undulated tubules associated with the endoplasmic reticulum and giant mitochondria with the centrally placed cristae were observed in occasional immunoblasts. Cytoplasmic fragments of immunoblasts and filamentous material among the cells were considered to correspond to the amorphous intercellular material seen in histologic sections.

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Human red cell membrane proteins were extracted by incubation of the ghost with hypotonic phosphate buffer (pH 7.4), N-ethylmaleimide and p-hydroxy-mercuribenzoate. In paroxysmal nocturnal hemoglobinuria (PNH), hereditary spherocytosis (HS) and hereditary elliptocytosis, the amount of proteins extracted by these procedures was significantly less than the amount extractable from the ghost of normal and aplastic anemia red cells. Polypeptide patterns of red cell membranes in these hematological disorders were essentially similar to those of normal ghosts. Analysis of the supernatant by SDS polyacrylamide gel electrophoresis revealed that this reduction was mainly due to the reduced amount of peripheral proteins extracted. The extraction of peripheral proteins by sulfhydryl reagents was accompanied by shape changes resulting in the formation of membrane vesicles, suggesting an important role of peripheral proteins in the maintenance of ghost shape. It is also suggested that qualitative abnormalities of peripheral proteins such as altered reactivity to sulfhydryl reagents and/or strong binding to the membrane are present in PNH, HS and hereditary elliptocytosis red cells.

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