aruyama H

Clinicopathological features of myeloid/natural killer (NK) cell precursor acute leukemia

Four patients (three males and one female) were diagnosed as myeloid/natural killer (NK) cell precursor acute leukemia in our department. Two patients showed the extramedullary involvement at initial presentation. Leukemic cells expressed CD7, CD33, CD45 and CD56 in all patients. Additionally, CD13, CD34, HLA-DR, cytoplasmic CD3 and myeloperoxidase were expressed in some patients. Trisomy 10 was found in two patients, which has not been reported in this disease. Therefore, myeloid/NK cell precursor acute leukemia might be rather heterogeneous especially in chromosomal abnormality though it seemed to constitute the distinct clinical entity among acute myeloid leukemia of M0 subtype.

Serum thrombopoietin levels in patients undergoing autologous peripheral blood stem cell transplantation

Recently, the ligand for c-mpl has been cloned and initial studies have shown it to be the platelet regulatory factor, thrombopoietin (TPO). To elucidate the role of TPO in the reconstitution of megakaryopoiesis and platelet production after stem cell transplantation, we measured serum TPO levels in nine patients undergoing autologous peripheral blood stem cell transplantation (PBSCT) and in healthy volunteers. Serum TPO levels significantly correlated with the degree of peripheral thrombocytopenia and a strong inverse correlation between serum TPO level and platelet count was observed (r = −0.700, P < 0.001). serum tpo levels began to rise as the platelet count decreased after chemotherapy. tpo levels peaked at over 25.00 fmoles/ml between days 0 and 10; tpo levels then decreased gradually as the platelet count began to rise. one patient with multiple myeloma received purified cd34+ peripheral blood stem cells. No difference was observed in the kinetics of serum TPO levels between unfractionated and purified PBSCT. These observations suggest that TPO plays a critical role in the reconstitution of megakaryopoiesis and platelet production after PBSCT.

Inversion of chromosome 3 in a case of chronic myelogenous leukemia with abnormal thrombopoiesis

A patient with chronic myelogenous leukemia (CML) associated with a remarkable increase of micromegakaryocytes in bone marrow was revealed to have an abnormality of a long arm of chromosome #3, i.e., inv(3)(q21q26), in addition to a complex Ph translocation: t(9;22;15)(q34;q11;q22). Although several cases of acute leukemia with inv(3)(q21q26) and abnormal megakaryocytes have been reported, this is the first case in which the association of inv(3)(q21q26) with a megakaryocytic abnormality was observed in a patient with CML. Our findings suggest that this structural rearrangement may be more specifically associated with abnormal thrombopoiesis than are other structural anomalies of 3q.

Translocations t(15;17) and t(9;14)(q34;q22) in a case of acute promyelocytic leukemia with increased number of basophils

A patient with a variant form of acute promyelocytic leukemia (M3 variant) associated with an increased number of basophils was found to present a reciprocal translocation, t(9;14)(q34;q22) in addition to t(15;17)(q22;q12). Although several cases of acute nonlymphocytic leukemia with increased bone marrow and peripheral blood basophils have been reported, this is the first case in which both the t(9;14) and basophilia were observed in a patient with M3. Our findings support the hypothesis that 9q34 may be associated with the chromosomal location of genes regulating the production and maturation of basophils.

Inflammatory pseudotumor of the spleen complicated by idiopathic thrombocytopenic purpura

Inflammatory pseudotumor is a benign disease entity of unknown etiology and pathogenesis, which mainly affects elderly patients and involves various parts of the body such as the orbit, respiratory tract, gastrointestinal tract, liver, or soft tissue. However, inflammatory pseudotumor of splenic origin has been rarely reported [1]. Here, we report a patient with splenic inflammatory pseudotumor which was simultaneously complicated by idiopathic thrombocytopenic purpura (ITP). In April 2002, a 68-year-old female was admitted to our hospital because of the bleeding tendency on her skin. She was not anemic, and neither lymph node swellings nor hepatosplenomegaly was found. Laboratory findings at that time were as follows: platelets 2×10/l, hemoglobin12.1g/dl, and white blood cells 8.1×10/l with normal differential counts. Coagulation test showed no significant abnormalities. Bone marrow was normocellular with increased number of megakaryocytes. She was serologically negative for human immunodeficiency virus (HIV). She was diagnosed as having ITP and received oral prednisolone (1.2 mg/kg). Both her platelet counts and bleeding tendency showed rapid recovery, and prednisolone was stopped in June 2002. Whereas routine ultrasonographic examination on her admission revealed the solitary hypoechoic mass (3 cm in diameter) in her spleen, no additional space-occupying lesions in the other organs or abdominal lymph node swellings were found. Concerning the splenic tumor, she had been asymptomatic and carefully observed for 10 months. However, her splenic tumor gradually enlarged and reached up to 5 cm in diameter in February 2003 (Fig. 1a). Therefore, she underwent diagnostic splenectomy. Macroscopically, her splenic mass showed a whiteyellowish cut surface with elastic firmness, which was well circumscribed but not encapsulated (Fig. 1b). Histologically, her splenic mass was composed of granulomatous nodules infiltrated by variable inflammatory cells such as lymphocytes or plasma cells. Multinuclear giant cells were also found (Fig. 1c, d). Special stains for acid-fast bacilli and fungi were negative; therefore, she was diagnosed as having splenic inflammatory pseudotumor. Epstein–Barr virus (EBV) was not detected in the tumor by in situ hybridization. She had been well without relapse of both ITP and inflammatory pseudotumor until November 2004. In the literature, most cases of splenic inflammatory pseudotumor were found incidentally, either at autopsy or during the work-up for other diseases. This is rare with only 82 reported cases in a recent literature survey [1]. Among these, there have been four reported patients (one male and three females with ages ranging from 20 to 65 years) with splenic inflammatory pseudotumor who concomitantly suffered from ITP [2, 3]. These findings suggested the autoimmune etiology of this disease. However, the clinical course of splenic inflammatory pseudotumor in our patient did not seem tobe in accordancewith that of ITP, namely, the splenic tumorhadgradually enlargedafter ITPwas alleviatedbyoral prednisolone. Alternatively, immunosuppressive therapy with prednisolone for ITPmight have triggered the progression of splenic inflammatory pseudotumor in our patient. Immunosuppressive therapy has been known to accelerate the disease progression in certain diseases such as EBVassociated post-transplant lymphoproliferative disorder. Of M. Hatsuse . S. Murakami . H. Haruyama Department of Medicine, Social Insurance Kyoto Hospital, Kyoto, Japan

Giant Neutrophils Derived from Tetraploid Leukemic Clone in an Acute Myeloblastic Leukemia: Cytofluorometric Study

Near-tetraploid chromosomes were observed in a patient with acute myeloblastic leukemia with maturation (M2 in FAB classification). Large and morphologically bizarre leukemic cells and giant neutrophils in each maturation stage were observed in both peripheral blood and bone marrow. Cytogenetic studies revealed that the main stem line was 93; XXYY, +9, and DNA cytofluorometry showed that these large leukemic cells and giant neutrophils had 4C DNA content. These findings strongly suggested that these giant neutrophils were derived from leukemic clone with tetraploidy.

Serial measurement of free light chain detects poor response to therapy early in three patients with multiple myeloma who have measurable M-proteins

Free light chain (FLC) assays are important in the diagnosis and monitoring of patients with multiple myeloma (MM). Serum FLC concentrations also correlate with disease course in the majority of MM patients and have been incorporated into the new response criteria. Although baseline values of FLC are prognostic in newly diagnosed MM, serial measurement of serum FLC does not appear to be of greater value in patients who have measurable M-proteins by electrophoresis. We examined the kinetics of serum FLC in six patients with newly diagnosed MM during treatment with high-dose dexamethasone (HD-DEX) and bortezomib and dexamethasone. In some cases, the involved serum FLC increased in the latter part of each chemotherapy cycle before the start of the next cycle, especially in HD-DEX, suggesting that the response to these agents may be insufficient for induction therapy for MM. Earlier disease assessment by serum FLC assays may be of value in detecting poorly responding patients who require alternative forms of therapy.

Chromosome 14 Abnormality with a Breakpoint of p12 in Adult T-cell Leukemia

We describe a patient with adult T-cell leukemia (ATL) with a 14p chromosomal abnormality. Cytogenetic study revealed two clonal populations of leukemic cells in the peripheral blood sample. Both clones were karyotypically related to each other. One of them showed rearrangement of chromosome 14 at break band p 12 (14p12) in addition to + 3, + 7, -X and del(6) (q14q21). The nucleolar organizer region (NOR) is assigned to the band 14p12 and the role of the rearrangement of chromosome 14p12 in the pathogenesis of ATL is discussed.