The immature information IMI channel on an SE-9000 automated hematology analyzer was used for detection of stem cells in cord blood and the results were compared with other standard methods, including flow cytometry analysis and progenitor assays. After the removal of red blood cells, the IMI count in cord blood samples significantly correlated with the number of CD34⁺ cells (r=0.810), CFU-GM (r=0.606) and BFU-E (r=0.961). The cell count in hematopoietic progenitor cell (HPC) area also showed a correlation with CD34⁺ cell number (r=0.722), but to a lesser extent than the IMI count; no significant correlations were observed with the numbers of progenitor cells. Cord blood contained higher fractions of CD34⁺/CD38^- and CD34⁺/CD117⁺ cells representative of immature subclasses of progenitor cells. This might explain the difference in the HPC module results for the cord blood and peripheral blood stem cell samples. A higher coefficient of correlation was obtained with leukocyte suspensions than with whole blood samples. These results demonstrated the usefulness of IMI-positive cell measurement as a stem cell screening test for cord blood banking.

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We evaluated measurements of PAIgG, reticulated platelets (RP), plasma thrombopoietin (TPO) levels, and platelet size to determine whether these parameters were useful for the differential diagnosis of idiopathic thrombocytopenic purpura (ITP), aplastic anemia (AA), and hypoplastic thrombocytopenia (HypoT). The percentage of RP (%RP) in patients with ITP was significantly higher (25.2±11.0%, P<0.001) than in normal subjects (7.9±2.8), and the sensitivity, specificity, and predictive value of %RP in diagnosing ITP were 82%, 95%, 96%, respectively. On the other hand, TPO levels in patients with AA and HypoT were significantly higher (355.5±218.7 pg/ml, P<0.001, and 376.4±347.2, P<0.001, respectively) than in normal subjects (36.7±23.0). The sensitivity, specificity, and predictive value of TPO in diagnosing AA and HypoT were 88%, 89% and 86%, respectively. We also sought to determine whether the simultaneous measurement of %RP and TPO improved their value in the differential diagnosis of ITP, AA, and HypoT. However, simultaneous measurement did not yield significant improvements in sensitivty, specificity, or predictive value. These results indicated that measurements of %RP will suffice for the diagnosis of ITP, and that measurements of TPO are adequate for the diagnosis of AA and HypoT.

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In December 1997, we conducted a nationwide survey of cases of primary allogeneic peripheral blood stem cell transplantation (allo-PBSCT) performed in Japan between December 1994 and November 1997 Data was collected on 103 patients with hematologic malignancies, aplastic anemia, or solid tumors. Eighty-seven patients received transplants from HLA-identical siblings, and 16 from HLA-mismatched related donors. Granulocyte-colony stimulating factor (G-CSF) mobilized peripheral blood stem cells (PBSC) were collected from donors by 1 to 3 aphereses. Apheresis products contained a median 5.4 (1.0-30.4)×10⁶ CD34+cells/kg. Most patients were given cyclosporine and methotrexate for graft-versus-host disease (GVHD) prophylaxis. Median days to ANC>500/μl and platelets>50,000/μl were 13 (7-49) and 16 (10-94), respectivcly. Grade II-IV acute GVHD developed in 37/99 (37.4%) and chronic GVHD in 59/86 (68.6%) patients. The incidence of treatment-related mortality within 100 days after transplant was 16.1%. Fifty-five patients (59.6%) were alive after a median follw-up of 794 days. More patients and longer follow-up periods will be required to assess the efficacy of allo-PBSCT and evaluate graft-versus-leukemia effect along with the incidence of acute and chronic GVHD.

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A 47-year-old woman presented with severe neutropenia accompanied by diplopia and orbital pain. Her bone marrow was normal except for the absence of segmented neutrophils. Because the administration of granulocyte colony-stimulating factor (G-CSF) at a dose of 1 μg/kg/day was not sufficiently effective and neutropenia developed, the patient was admitted to our hospital. Physical examination revealed painful ophthalmoplegia and hypoalgesia in the first region of trigeminal nerve, suggestive of Tolosa-Hunt syndrome. Severe neutropenia was observed in both peripheral blood and bone marrow, together with mild anemia and thrombocytopenia. The life span of red cells and platelets was shortened. High PAIgG levels, a positive Coombs test, and a positive test for anti-NA1 antibody suggested that blood cells were being destroyed by an autoimmune mechanism. Corticosteroid hormone therapy preceded by the administration of G-CSF at 5 μg/kg/day was effective for both neutropenia and in improving the patient's neurological findings.

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We encountered a 44-year-old woman with suspected chronic myelocytic leukemia (CML) in the acute phase that was difficult to be differentiate from Philadelphia chromosome (Ph)-positive acute lymphoblastic leukemia (ALL). At disease onset, her bone marrow showed an increase in blasts that were negative for myeloperoxydase (MPO) and Positive for CD10, 19, 34, and HLA·DR. Standard type Ph was detected by chromosome analysis, and both major and minor BCR/ABL m-RNA were detected by reverse-transcriptase polymerase chain reaction (RT-PCR) methods. Neutrophil alkaliphosphatase (NAP) score was normal, and neither eosinophilia nor basophilia was observed in peripheral blood. Under a presumptive diagnosis of Ph-positive ALL (L2), the patient was given AdVP (doxorubicin, vincristine, and prednisolone) therapy followed by a regimen of LMVP (L-asparaginase, mitoxantrone, and VP), and obtained a complete remission 2 months later. At that time, FISH analyses of her bone marrow and blood cells no longer detected bone marrow Ph or BCR/ABL fusion gene. A month later, however, the leukemia relapsed with an increase in MPO-positive blasts in bone marrow, and the patient died soon thereafter. We finally concluded that her leukemia was not Ph-positive ALL, but CML in the acute phase at disease onset.

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A 50-year-old man was admitted to our hospital because of intracranial hemorrhage and thrombocytopenia (platelet count: 3,000/μl). Low levels of IgG (76 mg/dl) and IgA (30 mg/dl) and a normal pattern of peripheral blood T and B cell subsets yielded a diagnosis of common variable immunodeficiency (CVID). The number of megakaryocytes in bone marrow was within normal limits (64/μl), and a diagnosis of idiopathic thrombocytopenic purpura was made. Both high-dose intravenous gamma-globulin and prednisolone were ineffective. Because of the coexistence of CVID, splenic irradiation (total 15 Gy) was performed instead of splenectomy. The platelet number began to increase 5 days after the initiation of irradiation, had increased to 8.7×10⁴/μl at the end of irradiation, and was 22.9×10⁴/μl 2weeks later.

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A 58-year-old man was referred to our hospital because of painful swelling in the left lower leg and leukocytosis in January 1999. Moderate hepatosplenomegaly but no lymph node swelling was observed. Marked leukocytosis (leukocytes 44.9×10⁴/μl with 95% morphologically prolymphocytes) and thrombocytopenia were detected. The surface phenotype of the leukemia cells was CD1^-2⁺3⁺5⁺7⁺4⁺8⁺25⁺. Magnetic resonance imaging revealed dilated veins in the left lower leg. An abnormal 47XY, +22 karyotype was detected in 1/20 cells. Tests for HTLV-I antibody were negative. A diagnosis of T-cell prolymphocytic leukemia (T-PLL) was made on the basis of data including cytochemical and electron microscopic findings. Although 2 courses of chemotherapy comprising vincristine, cyclophosphamide, and prednisolone improved the venous thrombosis in the leg, the leukemia cells were refractory to chemotherapy. To prevent the recurrence of venous thrombosis due to leukostasis, the patient underwent repeated leukapheresis. The leukocyte count was maintained at around 20.0×10⁴/μl after total 7 courses of leukapheresis, one course of which comprised 7l of extracorporeal circulation. In addition to the rare presentation of venous thrombosis, the CD4⁺8⁺25⁺ phenotype observed in this case is rare in patients with T-PLL.

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A 16-year-old girl who had been treated for B-precursor acute lymphoblastic leukemia (ALL) presented with a mass in the left orbit as an isolated relapse. Five years earlier, she had undergone chemotherapy that promptly resulted in a complete remission. For prophylaxis of central nervons system leukemia, the patient received cranial radiotherapy (18 Gy) with shielding of the orbit and 15 cycles of intrathecal MTX and prednisolone. Cerebrospinal fluid analysis did not detect infiltration during the treatment course. Exophthalmos of the eft eye and hyperemia of the conjuctiva developed 15 months after therapy. Computed tomography showed a retro-orbital mass behind the left eye. Though bone marrow aspiration was negative, biopsy specimens from the mass disclosed the infiltration of CD10-positive leukemic blasts. After 8 courses of chemotherapy, 5 cycles of intrathecal MTX, and orbital irradiation (24 Gy), the mass disappeared. All relapse as an isolated retro-orbital mass is very rare, and only 3 cases have been reported to date.

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A 59-year-old man was admitted in December 1995 because of general fatigue without lymphadenopathy. Increased abnormal lymphocytes (70%) were observed in peripheral blood. Bone marrow aspiration was a dry tap. Biopsy specimens revealed hypercellularity with infiltration of abnormal lymphocytes. Surface marker analysis of tumor cells was positive for CD5, CD19, CD20, HLA-DR, κ, and sIgM and negative for CD10. Cytogenetic analysis disclosed a complex abnormal karyotype including t(3;22) and rearrangement of the BCL6 gene. The patient was given a diagnosis of CD5 positive B-cell lymphoma, but died in January 1997 despite repeated chemotherapy. This case was unique because BCL6 rearrangement has been reported in various types of B-cell lymphoma but rarely associated with leukemic types without lymphadenopathy.

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Essential thrombocythemia (ET) was diagnosed in a 31-year-old woman who had a miscarriage in the first trimester of her first pregnancy. Because of her wish for child, the patient was given alpha interferon (α-IFN) instead of hydroxyurea or aspirin to lower her platelet count to about 80×10⁴/μl. She also had a miscarriage in her second pregnancy. Thereafter, the platelet count was kept at 60-70×10⁴/μl by dose escalation of α-IFN. In the 35th week of her third pregnancy, the patient delivered a healthy baby with a normal blood count. Several small infarctions were observed in the placenta. Control of platelet count by α-IFN administraion was effective in preventing recurrent miscarriage associated with ET, and had no adverse effect on patient fertility or fetal development.

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