Clinical evaluation of a combination treatment with cefminox and fosfomycin for infections complicated in patients with hematological disorders]

We evaluated clinical effects and toxicities of a combination treatment with cefminox (CMNX) and fosfomycin (FOM) for infections complicated with hematological disorders in 56 patients. Among those, 52 patients including 22 with malignant lymphoma, 19 with acute leukemia, and 11 with other hematological disorders were evaluable. Excellent and good responses were obtained in 33 (63.5%) of the 52 patients. This treatment was also effective in 5 of 9 cases in which granulocyte counts were less than 500/mm3 through the course of administration. Side effects were observed in only one patient. Mild nausea occurred but was not serious. These results indicate that the combination of CMNX and FOM is an effective and safe regimen for the treatment of infections complicated in patients with hematological disorders.     

Long-term survival of patients with biliary tract cancers with lymph node involvement

In extrahepatic biliary tract malignancies, the prognostic value of lymph node involvement remains unclear. For a total of 161 tumors of the gallbladder (GBC) and bile duct (BDC) resected between 1982 and 1992, the number and extent of metastatic nodes were investigated according to the location of the primary tumor. In GBCs and upper two-third BDCs, more than half of node-positive tumors involved multiple nodes (58% and 63%), and node-positive tumors were associated with a significantly poorer rate of patient survival compared with node-negative tumors. However, GBCs with one or two nodes involved resulted in a longer survival time than those with three or greater (P = 0.0045). In lower one-third BDCs, even patients with node-positive tumors showed a 60% 5-year survival rate. There were 15 long-term survivors (i.e., survived for more than 5 years after surgery) with node-positive tumors in the GBC group, 4 in the upper BDC group, and 6 in the lower BDC group. Thirteen GBCs had one or two involved nodes, and 12 of the 13 GBCs positive nodes were located within the hepatoduodenal ligament. In upper and lower BDCs, pericholedocal nodes and nodes around the pancreatic head respectively, were most frequently involved. We concluded that lymphatic spread is an important prognostic determinant in both GBCs and upper BDCs. The prognosis of GBCs is especially influenced by the number of nodes involved. In lower BDCs, lymphatic metastasis does not necessarily preclude long-term survival.     

A Case of Infantile Febrile Psoriasiform Dermatitis

Abstract: An infant suffered from psoriasiform dermatitis complicated by severe constitutional symptoms. During his 12 months of hospitalization, the symptoms exacerbated periodically despite numerous therapeutic trials. Histologie findings revealed lichenoid psoriasiform dermatitis with striking eosinophilic necrosis of epidermal cells and satellite cell necrosis. Immunohisfochemically, CD1 + Langerhans cells had almost disappeared, and CD8+ cytotoxic-suppressor T cells were predominant over CD4+ helper-inducer T cells in the epidermis. These findings in the skin biopsy specimens suggested some similarity to graft-versus-host disease but no known cause of that disorder was proved. Finally, methotrexate was effective. The patient became afebrile, and his skin lesions Improved, leaving almost no scarring. This patient seems to have had specific clinical features that do not correspond with any of the known dermatitites. Clinical, histoiogic, and laboratory findings did not uncover any etiologic factors.     

Coexistence of Generalized Morphea with Histological Changes in Lichen Sclerosus et Atrophicus and Lichen Planus

We reported a 44-year-old Japanese woman with generalized multiple sclerotic plaques, which showed hisological findings of morphea. This patient also had an erosive lesion on her mouth; its histological findings were consistent with lichen planus. A sclerotic lesion on her thigh showed the histological findings of lichen sclerosus et atrophicus (LSA). These data suggest that similar etiologic events or closely related pathologic processes are involved in morphea, lichen planus, and LSA.     

Pulmonary blastoma

We describe a resected pulmonary blastoma in an 84-year-old male, the oldest of previously reported patients. A chest X-ray showed a 2 cm-sized abnormal shadow in the left lung field. Five months later computed tomography demonstrated a well-demarcated heterogenous mass, measuring 12 cm in diameter, in the left lower lobe of the lung. This mass was diagnosed as a carcinoma using echo-guided percutaneous biopsy. The patient underwent a left lower lobectomy. The resected specimen revealed the tumor to be a pulmonary blastoma arising from lung tissue. Preoperative diagnosis of pulmonary blastoma is extremely difficult because of the histological heterogeneity of tumor. Since it has been noted that pulmonary blastoma rapidly progresses in a short period of time, surgical treatment should be undertaken as quickly as possible when such a tumor is suspected.     

Lymphoid tumor in the orbit: malignant or benign? MRI, histomorphological and molecular genetic analysis of eight cases

Differentiation between benign and malignant lymphoid infiltrates of the conjunctiva and the orbit is difficult even on the basis of histopathological appearance. We describe eight cases with low-grade malignant lymphoma, mucosa-associated lesion tissue (MALT) lymphoma. Laboratory analysis was done using microscopy, immunophenotyping and gene rearrangement using the Southern blot technique. DNA-rearrangement analysis allows a very precise determination of the benign and malignant lymphoid infiltrates of the ocular region. Our MRI findings show that these tumors can also generally be distinguished by their signal intensity on T2-weighted images. These findings might explain the favorable prognosis for MALT lymphoma of the conjunctiva and the orbit, and the absence of any recurrence in follow-ups ranging from 6 months to 9 years.     

Morphology and Morphometry of the Deformed Cervical Vertebrae in a Mutant Knotty-Tail (knt/knt) Mouse

Mice with short and knotty tails arose as a spontaneous mutant in an ICR strain, and they have been named knotty-tail mouse (gene symbol; knt). They also have a minor anomaly of the cervical vertebrae, especially in the axis. In this study, the cervical vertebrae of knotty-tail (knt/knt) mice were investigated by morphological and morphometric examinations during the prenatal and postnatal period. From the observation of double-stained preparations of knt/knt mice, morphological changes of cervical vertebrae were confined to the vertebral arch of the axis, which was asymmetrical and hypoplastic. From the morphometric analyses, outside of the axis, minor anomalies (i. e., broadened cervical vertebrae in the transverse direction, shortened and broadened ventral tuberculum of the atlas, thickened ventral lamina of 6th cervical vertebra) were maintained in the cervical vertebrae of knt/knt mice. Morphological deformity, reflecting an adult osseous anomaly, had been already formed in the cartilaginous axis prenatally. In the papain-digested preparations of knt/knt mice, a bony invagination into the canal was detected in the axis, and the morphometric analyses on axis revealed that the growth of spinous process was apparently disturbed in comparison with that of ICR mice.     

A case report of a 63-year-old patient with malignant hyperthermia]

We have experienced a case of fulminant malignant hyperthermia who was a 63-year-old female weighing 44 kg. There was no particular past history nor family history. She underwent right mastoidectomy because of chronic otitis media. Her preoperative physical status was ASA I. She was premedicated with diazepam 10 mg and loxatigine 75 mg P.O. The induction was done with thiamylal 200 mg IV and fentanyl 0.1 mg IV followed by vecuronium 6 mg IV for endotracheal intubation. Intubation was easy and uneventful. Anesthesia was maintained with nitrous oxide 3 l.min-1, oxygen 3 l.min-1 and enflurane 2.0%. Seventy min after the induction of anesthesia, arterial blood gas analysis showed severe respiratory acidosis (PCO2: 63.2 mmHg, pH: 7.27) and it was improved with manual hyperventilation at that time. Pulse rate increased from 80 to 115 b.p.m. 20 minutes later. Then, the patient was ventilated with 100% oxygen, and anesthetic circuits and machine were exchanged for new units. Surgery was postponed. Muscle stiffness of upper extremities was observed and her temperature increased to a maximum of 38.9 degrees C. Surface cooling was started and dantrolene sodium 60 mg and furosemide 20 mg were given intravenously. The patient was transferred to the intensive care unit, and clinical signs improved gradually within one hour. Serum enzymes; CPK, LDH, GOT and GPT increased on the first postoperative day. On the 11 th postoperative day skeletal muscle biopsy was done under local anesthesia with 1% procaine and Ca-induced Ca-release rate test revealed positive for enflurane. This is the oldest patient of malignant hyperthermia reported in Japan.     

Human common acute lymphoblastic leukemia-derived cell lines are competent to recombine their T-cell receptor delta/alpha regions along a hierarchically ordered pathway.

Rearrangements of the T-cell receptor (TCR) delta locus are observed in the majority of human B-cell precursor acute lymphoblastic leukemias (ALL) with a striking predominance of V delta 2(D)D delta 3 recombinations in common ALL (cALL) patients. Recently, we and others showed that almost 20% of cALL cases are characterized by further recombination of V delta 2(D)D delta 3 segments to J alpha elements, thereby deleting the TCR delta locus in analogy to the delta Rec/psi J alpha pathway in differentiating alpha/beta-positive T cells. We report here that two human cALL-derived cell lines, REH and Nalm-6, are competent to recombine the TCR delta/alpha locus under standard tissue culture conditions. Analysis of different REH subclones obtained by limiting dilution of the initial culture showed a biased recombination of V delta 2D delta 3 to distinct J alpha elements. During prolonged tissue culture, a subclone acquired growth advantage and displaced parental cells as well as other subclones. Frequently, the DJ junctions of REH subclones contained extended stretches of palindromic sequences derived from modified D delta 3 coding elements. The other cell line, Nalm-6, started the TCR delta/alpha recombination with an unusual signal joint of a cryptic recombinase signal sequence (RSS) upstream of D delta 3 to the 3' RSS of D delta 3. The RSS dimer was subsequently rearranged in all investigated subclones to an identical J alpha element. Both cell lines might become valuable tools to unravel the complex regulation of TCR delta/alpha recombination pathways in malignant and normal lymphopoiesis.