The hypereosinophilic syndrome and lymphoblastic leukemia with extra C-group chromosome and q14+ marker.

Hypereosinophilic syndrome is probably a disease of diverse etiologies. We studied the bone marrow of a patient with HES and found a population of L-1 lymphoblasts. These cells failed to grow in double-layer agar cultures, were "null cells," and contained a 14q+ marker chromosome consistent with a malignant lymphoproliferative disorder. Complete remission was induced with vincristine, prednisone, and L-asparaginase but the patient died from the consequences of cardiac fibrosis. An underlying lymphoproliferative process should be carefully sought in patients with HES to afford the best opportunity for cure.

Septicemia in association with acute lymphoblastic leukemia.

Fifty consecutive episodes of septicemia were studied in 41 children who had acute lymphoblastic leukemia. Seventy-six percent of these episodes occurred when the absolute granulocyte count was 200/mm3 or less and were caused by gram-negative enteric and gram-positive mucocutaneous bacteria. In eight patients, Streptococcus pyogenes was isolated at the time when ALL was diagnosed. Multiple anaerobic and aerobic isolates from a single blood culture were associated with abdominal distress, whereas Streptococcus pneumoniae and Hemophilus influenzae septicemia occurred in associated with respiratory illnesses. When patients with severe compromise of anatomic barriers or respiratory disease were excluded, 94% of all patients with septicemia had an AGC of less than 200/mm3. The data provide guidelines for treatment for febrile patients with ALL based upon the AGC, the phase of the disease, and on the presence of associated respiratory or abdominal findings.

Glucose phosphate isomerase deficiency with hereditary nonspherocytic hemolytic anemia.

Eight children (5 living, 3 deceased) with severe hereditary nonspherocytic hemolytic anemia caused by glucose phosphate isomerase deficiency have been observed in two Kentucky and Indiana families. All affected children were severely anemic in early life. Three deaths occurred in young patients who did not receive adequate transfusions of blood or whose parents refused to permit splenectomy. Splenectomy generally abolishes the requirement for blood transfusion. No patient has required regular transfusion of blood after puberty. Growth and development have been surprisingly normal and no patient has died of infection. The anemia is expressed as an autosomal recessive trait, but the enzyme variant can be detected in hematologically normal heterozygotes. The abnormal isomerase molecule is heat labile and is contained in neutrophils and lymphocytes as well as in erythrocytes.