A case of an unusual type of hepatoblastoma in a 5‐month‐old male infant is described. The tumor showed the following unusual features as compared with the epithelial and mixed types of hepatoblastoma: (1) The tumor cells presented a primitive anaplastic appearance without any resemblance in terms of cytologic features or arrangement to embryonal or fetal liver. (2) Abundant mucoid material containing acid mucopolysaccharide was present giving a slimy, gelatinous, gross appearance to the tumor and its metastases. (3) Well‐defined tubular structures were present in some foci within the tumor parenchyma. (4) The tumor resulted in a rapidly fatal course, with metastases to the lungs and widespread peritoneal seeding despite complete surgical resection of the primary tumor. Yolk sac carcinoma and undifferentiated (embryonal) sarcoma of the liver were considered in the differential diagnosis. On electron microscopic examination, the tumor cells showed cytoplasmic features and junctional complexes consistent with their epithelial origin. Alpha‐fetoprotein in the blood, which was markedly elevated prior to surgery, returned to a normal level postoperatively. Hepatoblastoma with the combination of features described above has not been previously reported. Because of the two striking and easily recognizable features viz. total lack of differentiation of tumor cells and presence of abundant mucoid material, the authors designated the tumor as mucoid anaplastic hepatoblastoma.

Reports of acute nonlymphoblastic leukemia occurring after successful treatment of Hodgkin and non-Hodgkin lymphoma (NHL) are appearing with increasing frequency. Two years after completion of LSA2-L2 therapy for stage III, poorly differentiated lymphocytic lymphoma, a 16-year-old boy developed a preleukemic state characterized by a refractory macrocytic anemia with excess blasts, dyshematopoiesis, abnormal cluster:colony ratio on in vitro bone marrow culture, and acquired deficiencies of erythrocyte pyruvate kinase, triose phosphate isomerase, and adenylate kinase. Four months later acute myeloblastic leukemia was evident. The RNA index determined by flow cytofluorometry was increased. Four marker chromosomes were found and involved complex translocation of chromosomes 11 and 17 (t11;l17) in 100% of the cells, and chromosomes 4 (t4q;4) in 10% of the cells. A thorough literature search uncovered four other reports of acute nonlymphoblastic leukemia occurring in children treated for NHL and a total of 58 cases in the adult and pediatric age groups. Over 50% of the patients had AML, were mean over 50 years of age, and were treated with radiotherapy and chemotherapy. It is anticipated that additional cases of second malignancies will be reported in this population of patients whose outlook for the curability of the primary malignancy is 75%.

The immunoregulatory activity of bone marrow leukemic blasts from five patients with null-cell acute lymphoblastic leukemia (ALL) and two children with T-cell ALL was evaluated. Blasts were studied in co-culture for their effects on the proliferative responses of normal allogeneic peripheral blood lymphocytes to phytohemagglutinin. Mitomycin C-treated null cell ALL blasts from two of five children suppressed the proliferative responses of normal responder cells by 80% and 58% whereas those from two other children enhanced the responses by 118% and 31%. T-cell leukemic blasts from one patient with T-cell ALL exhibited helper cell activity (26%) and T-leukemic blasts from the other demonstrated suppressor cell activity (53%). The helper cell activity of leukemic blasts was associated with stimulating capacity of null blasts in one-way mixed lymphocyte reactions. In six of seven cases, incubation of blast cells with concanavalin A (20 microgram/ml) for 48 hours prior to co-culture with responder cells did not result in the generation of significant suppressor cell activity. Our study suggests that leukemic blasts from certain patients with 'null' and T-cell ALL may possess spontaneous helper or suppressor cell immunoregulatory activity. This functional heterogeneity of leukemic blasts may help in subclassifying the ALL.

No abstract

SCID i s a heterogeneous d i s o r d e r o f t e n a s s o c i a t e d with lymph-The unusual occurrence of qroup B streptococcal (GBS) disease in openia; hypothesized t o be due t o d e f e c t i v e l v m~h o i d d i f f e r e n t i asiblings lead to immune studies b f a boy whomimicked his older sister in developing septicemia and meningitis from GBS by the 4th day of life. His serum IgG level was only 40 mg%. His IgM was 36 and IgA was < 7. Maternal levels were all low: IgG 40, IgM 36, IgA 14 and IgE