ケツエキ シッカン カンジャ ノ テツカジョウショウ ニ タイスル ヒョウカ ホウホウ ノ ケントウ MRI ニヨル ヒ シンシュウテキ テツリョウ テイリョウ ソクテイ

【背景】血液疾患では長期輸血等で鉄過剰となる。肝鉄量は鉄過剰の重要な指標であり、欧米では MRI によ る非侵襲的定量法が普及しつつあるが、日本では殆ど行われていない。【方法】Gandon らの論文に基づき、腹部 MRI をグラディエントエコー法(GRE)で撮影、肝臓(L)と脊筋(M)の信号強度比(LBackground: Iron overload is a major problem for patients with hematological diseases. Liver iron concentration (LIC) is an important marker of iron overload. LIC has been measured by MRI (Magnetic Resonance Imaging) instead of invasive hepatic biopsy in European countries, but not yet in Japan. Methods: LIC was measured by MRI according to Gandon\u27s method (Lancet 2004;363:357-362) using gradient echo sequence (GRE). Signal intensity ratio of the liver and muscle (L/M) was measured. LIC was estimated from the linear correlation curve of L/M and LIC shown in Gandon\u27s paper. LIC estimated from MRI (LIC-MRI) was compared with serological markers such as serum ferritin level. Patients:Three patients with hematological diseases (post-transfusion iron overload, chronic hemolytic anemia, secondary myelofibrosis) and three healthy persons as normal controls. Results: LIC-MRI and serological markers were normal among the three normal controls. Two patients showed ten times higher LIC-MRI and ferritin levels than normal ranges. Conclusion: LIC-MRI showed an excellent correlation with serum ferritin level. Because of low examination numbers for the present cases, further study is necessary

フローサイトメトリー ニ ヨル ケッショウバン ハッケッキュウ ギョウシュウ ト カッセイカ ケッショウバン ノ カイセキ

Thrombosis is one of the most important health problems among developed countries. Roles of Platelet-leukocyte interaction in thrombosis has been gradually recognized. Increase in platelet-leukocyte aggregate has been reported among patients with acute myocardial infarction and cerebral infarction. Increase in platelet-leukocyte aggregate has also been reported among patients with myeloproliferative neoplasms, for whom thrombosis is the major clinical problem. Platelet-leukocyte aggregate may be useful as a marker of thrombosis risk. We measured platelet-leukocyte aggregate and platelet activation among healthy volunteers using flow cytometry. Percentage of platelet-monocyte aggregate was higher than that of platelet-granulocyte aggregate, and showed larger individual variation. Percentage of activated platelet showed better correlation with percentage of platelet-monocyte aggregate than with platelet-granulocyte aggregate. Percentage of platelet-leukocyte aggregate changed widely during storage periods

Two Cases of Primary Cold Agglutinin Disease Associated with Megaloblastic Anemia

We report two cases of primary cold agglutinin disease (CAD) associated with megaloblastic anemia in Japanese elderly patients. Case 1 was a 67-year-old male and Case 2 was a 55-year-old male. Both patients were diagnosed with primary CAD, with continuously high cold agglutinin titers (1 : >8,192 and 1 : 16,834, resp.), monoclonal IgM-kappa light chains, and no underlying disease. In addition, both patients had megaloblastic anemia due to vitamin B12 deficiency. One patient received rituximab and both received vitamin 12 supplementation. To date, no cooccurrence of primary CAD and megaloblastic anemia has been emphasized. Thus, the association of these hematological diseases may be incidental; however, given that CAD is an autoimmune disease which may show antibodies against intrinsic factor and gastric parietal cells, this association was thought to be probably not a coincidence. Clinicians should be aware of the possible simultaneous presence of autoimmune hemolytic/megaloblastic anemia in patients with primary CAD

FLT3/ITD Associated with an Immature Immunophenotype in PML-RARα Leukemia

Acute promyelocytic leukemia (APL) is characterized by the specific PML-RARa fusion gene resulting from translocation t(15;17) (q22;q12). Internal tandem duplication (ITD) of the FLT3 gene has been observed in approximately 35% of APLs, and large-scale studies have identified the presence of ITD as an adverse prognostic factor for acute myeloblastic leukemia (AML) patients. Aberrant expressions of surface antigens, such as CD2, CD34, and CD56, have been found in APL, but the implications of this are not well understood. We investigated the incidence of the FLT3/ITD mutation and FLT3/D835 (I836) point mutation in 25 APL patients. Incidence ratios of FLT3/ITD, D835 (I836), and both FLT3/ITD and D835 (I836) were 36%, 36% and 8%, respectively. FLT3/ITD+ cases showed a predominance of the bcr3 isoform (P = 0.008) and M3v morphology (P < 0.001). We found that all FLT3/ITD+ cases expressed CD2 (9 of 9) more frequently than that of FLT3/ITD− (1 of 16) (P < 0.001), while only one of the CD2+ cases (1 of 10, 10%) did not harbor FLT3/ITD, and all CD2+CD34+ cases (5 of 5, 100%) harbored FLT3/ITD. In addition, quantitative polymerase chain reaction analysis showed that FLT3 mRNA was more abundantly expressed in FLT3/ITD+ than that in FLT3/ITD− (P = 0.025), while there was no difference between D835(I836)+ and D835(I836)− with regards to aberrant surface-antigen expression, expression levels of FLT3 mRNA, M3v morphology, and the bcr3 isoform of PML-RARa mRNA. This study demonstrates that the presence of FLT3/ITD, but not D835 (I836), is closely related to aberrant CD2 expression and high expression levels of FLT3 mRNA. Our findings also suggest that FLT3/ITD as a secondary genetic event may block differentiation at the immature stage of APL

A case of acute promyelocytic leukemia with morphologic multilineage dysplastic changes

Although reports of typical acute promyelocytic leukemia (APL) cases rarely mention dysplastic changes, this report concerns a rare case of APL with tri-lineage dysplastic changes resembling the characteristic features of myelodysplastic syndrome (MDS). The patient, a 77-year-old Japanese male, was diagnosed as having pancytopenia with hematologic morphological abnormalities comprising micromegakaryocytes, neutrophils with hypo-granulation and negative peroxidase activity, and erythroblasts containing nuclei with abnormalities such as karyorrhexis. Although there is one report of a case of transformation of de novo MDS into APL and several reports of cases of therapy-related MDS transformed into APL, our patient had no history of cytopenia or of either chemo or radiation therapy. Our case can thus be considered to constitute a rare case of APL with dysplastic morphology