We describe a 14-year-old boy who exhibited left palatine tonsillar enlargement after 6 cycles of aggressive chemotherapy for diffuse large B-cell lymphoma of the right palatine tonsil. The cervical computed tomography scan at 4 months after completion of chemotherapy revealed enlargement of the left palatine tonsil in addition to the thymus without any clinical symptoms. The F-fluorodeoxyglucose positron emission tomography indicated focal areas of strong F-fluorodeoxyglucose uptake in the left palatine tonsil. Histologic examination confirmed tonsillar hyperplasia with no evidence of recurrence. Reactive tonsillar hyperplasia after chemotherapy is rarely reported.
This study suggests the importance of the NUB domain in host defense against mycobacteria. The unique presenting features in our patient indicate that a hypomorphic NEMO mutation can be associated with atypical pathological findings of the epithelial tissues in patients with BCG infection.
Histiocytic sarcoma, a rare hematopoietic neoplasm with evidence of histiocytic differentiation, is often refractory to conventional chemotherapy and radiotherapy, and its prognosis is generally dismal. The optimal management of this malignancy has not been established. We report a case of 8-year-old girl with histiocytic sarcoma involving the left femur. The tumor rapidly responded to a combination of cladribine and high-dose cytosine arabinoside, an aggressive salvage regimen for refractory Langerhans cell histiocytosis, and became impalpable during the first cycle. The patient has remained in complete remission more than 7 years from diagnosis.
To clarify the mechanism of progression and acquired drug resistance of leukemia, we searched for an overexpressed gene in drug-resistant leukemia cells and identified an approximately 5-kb transcript by using the subtraction method. The nucleotide sequence of the gene was highly homologous to those of human endogenous retrovirus (HERV) transcripts. Reverse transcriptase-polymerase chain reaction (RT-PCR) revealed that the gene was overexpressed in cells from 6 childhood acute lymphoblastic leukemia patients (60%) but not in bone marrow cells at remission. Peripheral blood mononuclear cells from normal controls (n=11) and bone marrow cells from non-leukemia patients (n=13) did not express the gene. These findings indicate that the gene may play a role in leukemogenesis and may be a novel leukemia marker. Further studies on the functional role of the gene are needed.
Objectives One of the reasons as to why chimeric antigen receptors (CAR)‐T cell therapy for malignancies other than CD19‐ or BCMA‐positive tumors has yet to produce remarkable progress is the paucity of targetable antigens. NKp44 is only expressed by activated natural killer cells and detects a variety of transformed cells, while it reportedly does not react with normal tissues. The aim of this study is to develop CAR‐T cell that can target multiple types of tumor cells. Methods We created a series of novel CAR constructs in first‐generation (1G) and second‐generation (2G) CAR format with the extracellular immunoglobulin‐like domain of NKp44 (NKp44‐CAR). Results Transduction of the best 1G construct into human primary T cells led to specific cytotoxic effects and cytokine secretion upon encountering multiple types of neoplastic cells including AML, T‐ALL and childhood solid tumors. Replacement of the extracellular hinge domain of NKp44 with that of CD8α resulted in diminished CAR function. The 1G NKp44‐CAR‐T cells exhibited significantly better tumor control in long‐term co‐culture assays compared with activated NK cells, as well as with NK cells transduced with identical NKp44‐CAR. T cells transduced with the best 2G‐CAR construct with 4‐1BB co‐stimulatory domain proliferated at significantly higher levels upon single antigen exposure and showed significantly better tumor control compared with the 1G‐CAR and 2G‐CAR with CD28 co‐stimulatory domain. Conclusions NKp44‐based CAR endows T cells with NK cell‐like anti‐tumor specificity. The CAR gene created in this study will be useful for the development of novel gene‐modified T‐cell immunotherapy.
A previously healthy, 8-month-old boy suffered from Kawasaki disease with rhabdomyolysis.Although Kawasaki disease (KD) is a systemic vasculitis affecting multiple organ systems, muscle involvement has rarely been reported [1,2,3,4,5,6]. This report describes rhabdomyolysis in a patient with KD.A previously healthy, 8-month-old boy was referred to another hospital because of a 5-day history of highgrade fever, cervical lymph node swelling, and exanthema. The patient was transferred to our hospital because of elevated creatine kinase (CK) levels. The patient did not take any anti-inflammatory drugs including ibuprofen before admission. Upon admission, physical examination revealed bilateral conjunctivitis, a strawberry-like tongue, enlarged cervical lymph nodes, polymorphic truncal rash and oedema of the peripheral extremities. His vital signs were as follows: body temperature 40.3°C; blood pressure 102/ 48 mmHg; and pulse rate 140 beats/min. The body weight was 9.6 kg (-0.4 kg before present illness). The patient did not suffer from apparent muscle pain, tenderness or weakness. Neurological examinations showed no remarkable findings. Laboratory studies revealed anaemia (red blood cell count 364·10 4 /ll, haemoglobin 9.3 g/dl, and haematocrit 28.9%), elevated C-reactive protein (CRP) (5.34 mg/dl, reference range 0.01-0.31 mg/dl), increased levels of aspartate aminotransferase (AST, 528 IU/l, reference range 11-31 IU/l), alanine aminotransferase (ALT, 225 IU/l, reference range 7-42 IU/l) and lactate dehydrogenase (LDH, 1,716 IU/l, reference range 232-427 IU/l), and an elevated CK level (10,356 IU/l, reference range 48-280 IU/l) with 98.6% MM isozyme. Serum concentrations of aldolase (25.0 IU/l, reference range 2.5-5.8 IU/l) and myoglobin (380 ng/ml, normal <60 ng/ml) were also elevated. Serum sodium (138 mEq/l), potassium (4.7 mEq/l), chloride (102 mEq/l) and creatinine (0.2 mg/dl) and blood urea nitrogen (4.9 mg/dl) were normal. Urinalysis showed 1+ protein and 1+ occult blood without red blood cells. Urinary concentration of myoglobin (111 ng/mL, normal <10 ng/ml) was elevated. Urinary electrolytes were as follows: sodium 10 mEq/l, potassium 9.1 mEq/l and chloride 17 mEq/l. Urinary creatinine concentration was 15.3 mg/dl. Antinuclear antibody, hepatitis B virus surface antigen, and anti-hepatitis C virus antibody were negative. Bacterial and viral culture of throat swabs grew no pathogens. Echocardiograms and electrocardiograms revealed no abnormalities. The patient was diagnosed as having KD with rhabdomyolysis. Intravenous infusion of 1 g/kg c-globulin for 2 days and oral administration of flurbiprofen (2 mg/kg per day) promptly improved his condition. His body temperature normalised 4 days after the start of treatment and his serum CK and CRP levels normalised 7 and 10 days after treatment, respectively. He was discharged with elevated AST, ALT and LDH levels 13 days after he was admitted to the hospital. The levels of these enzymes returned to normal 4 weeks later. A follow-up examination 4 months later showe...
Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with massive lymphadenopathy. We here describe RDD of a neonate who presented with paleness and hepatosplenomegaly but not lymph-node swelling. Routine laboratory studies showed anemia, thrombocytopenia, and an elevated value of gamma-glutamyl transpeptidase. Histological examination of the liver revealed a proliferation of histiocytes with abundant eosinophilic cytoplasm, which were positive for S-100 protein and CD68 but not CD1a and did not reveal Birbeck granules. Radiological studies showed hepatosplenomegaly and a narrowing of the hepatic vein, which might have contributed to hypersplenism resulting in anemia and thrombocytopenia. This case is thought to be congenital RDD without lymphadenopathy.
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