Diffuse large B-cell lymphoma with hemolytic crisis developed twenty years after the onset of Evans syndrome.

A 65-year-old woman was diagnosed with Coombs-positive autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) in May 1992. One month later, her PRCA went into remission following treatment but she developed idiopathic thrombocytopenic purpura and was diagnosed with Evans syndrome. Although her condition resolved with administration of prednisolone and azathioprine, it was necessary to continue treatment with gradual tapering over the following two decades. In October 2012, her hemolytic anemia again worsened, and lymph node swelling, splenomegaly and B symptoms developed. She was diagnosed as having diffuse large B-cell lymphoma (DLBCL) based on lymph node biopsy. However, AIHA was not considered to be the cause of her hemolytic anemia, but rather to be related to DLBCL. This was because a Coombs test and other extensive investigations for Coombs negative-AIHA yielded negative results. The patient underwent CHOP therapy, and all of her symptoms improved. Herein, we report this rare case in which DLBCL developed after the onset of Evans syndrome.

Cerebral toxoplasmosis after umbilical cord blood transplantation diagnosed by the detection of anti-toxoplasma specific IgM antibody in cerebrospinal fluid.

Cerebral toxoplasmosis is a rare, potentially fatal, complication of hematopoietic cell transplantation. Early definitive diagnosis is very difficult and it may be associated with a poor prognosis. Herein, we describe a 60-year-old woman who developed cerebral toxoplasmosis after cord blood transplantation for myelodysplastic syndrome. During treatment with tacrolimus and methylprednisolone for relapsed grade 2 acute gut GVHD, fever and disturbance of consciousness occurred on day 210. Brain MRI showed multiple ring-enhancing nodular lesions in the thalamus, basal ganglia, brainstem, and subcortical white matter. Cerebrospinal fluid (CSF) assessment revealed elevations of both anti-to-xoplasma IgM and IgG, which were also elevated in serum, but no evidence of other infections or malignancies. Notably, the IgM level was higher in the CSF than in serum. Thus, cerebral toxoplasmosis was diagnosed. Soon after administration of oral sulfamethoxazole/trimethoprim and intravenous clindamycin in combination with short-term dexamethasone for the cerebral edema, her symptoms and signs began to improve. On day 229, both IgM and IgG titers in CSF had clearly decreased but remained essentially constant in serum. She was discharged without clinically significant neurological disorders. This case suggests that CSF specific anti-toxoplasma IgM titers might be useful for early diagnosis of cerebral toxoplasmosis after transplantation.

[Usefulness of measuring serum procalcitonin levels by immunochromatographic assay in febrile neutropenia].

In order to clarify the usefulness of measuring procalcitonin (PCT) values under the extreme condition called febrile neutropenia (FN), PCT was measured with immunochromatographic assay (ICA) and electro-chemi-luminescence immunoassay (ECLIA) at two time points: upon FN occurrence and 12 to 24 hours after FN occurrence, and correlations and associations between the two methods were reviewed. A strong correlation between the ICA and ECLIA results was observed when Spearman's rank correlation coefficient was 0.878, and the association was also demonstrated by Fisher's direct test since P=4.68×10(-10). Special equipment is not required, the operations are simple, and the ICA method currently adopted by many facilities can be used as the standard method even for the clinical condition known as FN.

[Angioimmunoblastic T cell lymphoma complicated with endocapillary proliferative glomerulonephritis].

A 30-year-old female developed fever and multiple lymphadenopathy in September 2011. Her symptoms improved with antibiotic treatment. However, she again presented with fever and multiple lymphadenopathy in December 2011. In addition, she suffered from nephrotic syndrome with severe edema. She was therefore hospitalized to undergo detailed examinations. Renal biopsy revealed endocapillary proliferative glomerulonephritis. Since her renal function deteriorated rapidly, she was given steroid pulse therapy with methylprednisolone, followed by maintenance therapy with prednisolone. After treatment, her renal function improved but multiple lymphadenopathy persisted. Biopsy of a left axillary lymph node was then performed and revealed angioimmunoblastic T-cell lymphoma (AITL). She received CHOP therapy but showed no response. Therefore, she was given ESHAP therapy. A partial response was achieved and the nephrotic syndrome also resolved completely. We report this extremely rare case of renal dysfunction due to endocapillary proliferative glomerulonephritis complicated by AITL.

[Acute myeloid leukemia with monosomy 7 and inv(3)(q21q26.2) complicated with central diabetes insipidus].

A 20-year-old female presented with thirst, polyposia, and polyuria and was referred to our hospital because of leukocytosis and anemia. Bone marrow aspiration revealed 66.8% myeloperoxidase-positive blasts and trilineage myelodysplasia. The karyotype was 45, XX, inv(3)(q21q26.2), -7[19]. Therefore, a diagnosis of AML with inv(3)(q21q26.2) complicated by -7 was made. Moreover, hyposthenuria and a low anti-diuretic hormone (ADH) level were observed. Although cerebrospinal fluid analysis was normal, magnetic resonance imaging (MRI) revealed the absence of hyperintensity in the neurohypophysis in T1-weighted images. Therefore, she was also diagnosed with diabetes insipidus. After she was administered a desmopressin nasal spray, the volume of urine produced decreased. Following treatment with second induction therapy containing high-dose cytarabine for AML, she achieved complete remission in the bone marrow. Moreover, when the abnormality on MRI and the volume of urine were normalized, she discontinued desmopressin. Although diabetes insipidus is a rare complication of AML, the majority of AML patients who have diabetes insipidus have the abnormal karyotypes with inv(3)(q21q26.2)/t(3;3)(q21;q26.2) and monosomy 7. Further study is required to clarify the pathogenesis and develop a strategy for the treatment of this category of AML.

Prolonged sinus tachycardia caused by human herpesvirus 6 (HHV6) encephalomyelitis after allogeneic bone marrow transplantation.

A 19-year-old man with Philadelphia chromosome-positive acute lymphoblastic leukemia received an allogeneic hematopoietic cell transplant with unrelated bone marrow. On day 20, the patient developed impaired consciousness and disorientation. Examination of the cerebrospinal fluid showed 2×10(4) copies/mL of HHV6B. HHV6 encephalitis was diagnosed, as had HHV6 myelitis based on symptoms that included lancinating pain/pruritus in the lower limbs and dysuria/dyschezia. Concurrently, he showed sinus tachycardia. Even after clearance of the HHV6 genome from the plasma and CSF was achieved by treatment with foscarnet, sinus tachycardia persisted for another 100 days. We suspected prolonged sinus tachycardia due to dysautonomia caused by HHV6 encephalomyelitis.

Intravascular large B-cell lymphoma diagnosed by FDG-PET/CT and endometrial biopsy.

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin's lymphoma characterized by a proliferation of tumor cells within the lumina of small to medium-sized vessels. Because there are few or no concomitant solid lesions, a diagnosis of IVLBCL usually cannot be established by CT or MR imaging. Herein, we describe a case of IVLBCL involving the uterus, in which (18)F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) was useful for diagnosis. A 47-year-old woman was referred to our hospital because of fever and anemia. Laboratory examination demonstrated anemia and thrombocytopenia. Bone marrow aspiration and biopsy showed hemophagocytosis without involvement of lymphoma cells. Random skin biopsy did not demonstrate lymphoma involvement. FDG-PET/CT imaging showed FDG accumulation in the uterus. MR imaging demonstrated uterine leiomyoma only. Based on these findings, uterine endometrial biopsy was performed and histological diagnosis of IVLBCL involving the uterus was established. She received 6 courses of R-CHOP therapy and high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation. At present, she remains in complete remission after 33 months.

Two-photon absorption properties of Dehydrobenzo[12]annulenes and hexakis(phenylethynyl)benzenes: effect of edge-linkage.

Two-photon absorption (TPA) properties of two trefoil-shaped compounds with different edge linkages--tris(hexadehydrotribenzo[12]annulene) and tris(tetradehydrotribenzo[12]annulene)--and three asterisk-shaped compounds having no edge-linkage--hexakis(phenylethynyl)benzenes--are investigated experimentally by the open-aperture Z-scan and TPA-induced fluorescence methods with wavelength tuneable femtosecond pulses. The compound with ethynylene edge-linkage exhibits the most intense TPA (the maximal TPA cross section is 1300+/-170 GM at 572 nm where 1 GM=10(-50) cm(4) s molecule(-1) photon(-1)). The TPA activity of the compounds is primarily explained in terms of the planarity of the molecules in relation with the type of edge-linkage.

Synthesis and properties of trefoil-shaped tris(hexadehydrotribenzo[12]annulene) and tris(tetradehydrotribenzo[12]annulene).

[reaction: see text] Trefoil-shaped tris(hexadehydrotribenzo[12]annulene) possessing a substructure of the ultimate two-dimensional C(sp)-C(sp(2)) network, graphyne, and the related tris(tetradehydrotribenzo[12]annulene) were synthesized, and their ground- and excited-state properties were investigated.