Lymphoblastic crisis of chronic myelogenous leukemia. Hand mirror variant

We present, to our knowledge, the first extensively studied case of lymphoid L2 blast crisis of chronic myelogenous leukemia with a hand mirror cell (HMC) variant. Special stains revealed the leukemic cells to be terminal deoxynucleotidyl transferase positive by immunofluorescence and cytochemically positive for alpha-naphthyl acetate esterase and acid phosphatase (diffuse granular). Immunophenotyping identified the major leukemic cell population as B-cells that expressed CD10+, CD19+, and HLA-DR+. It was not possible to separate the HMC and the non-HMC leukemic population by gating various cell populations, dual staining, cytochemistry, or by terminal deoxynucleotidyl transferase. Gene rearrangements were observed in both Ig heavy-chain alleles and one T-cell antigen receptor gamma-subunit allele. The rearrangements occupied all of the cells, indicating that the HMC and non-HMC were of a common clonal origin. The patient had a mosaic karyotype, with 90% of the cells having t(9;22), t(8;14), and t(9;15) translocations, an additional chromosome 8, and deleted chromosomes 9 and 15. Antibodies to simian sarcoma-associated virus and baboon endogenous virus were isolated in the patient's peripheral blood plasma.     

Outcome for older burn patients

BACKGROUND: Physicians will be increasingly responsible for an aging society whose members demonstrate a notable striving for independence. HYPOTHESIS: With standard treatment of burns, older patients will have a survival rate of more than 70%, with at least 60% of patients becoming fully functional 6 months after hospital discharge. METHODS: A 7-year retrospective medical review of burn unit patients was performed, and 221 ( 11%) of 1957 patients who were at least 59 years old were identified. RESULTS: Of 97 women (44%) and 124 men (56%), 64 (29%) had an associated smoke inhalation injury; 146 (66%), flame injury; and 44 (20%), scald injury. The bedroom and/or living room were the most common areas of injury (90 [41%]), followed by outdoors and the workplace (62 [28%]), the kitchen (40 [18%]), the bathroom ( 18 [8%]), and the garage or basement (11 [5%]) (P<.005). One hundred twenty-six injuries (57%) were associated with impaired judgment, mobility, or both. On hospital admission, 74 patients (36%) were intubated, 60 (30%) required intubation postoperatively, and 34 (18%) required both. The survival rate was 159 patients (72%) overall. Findings from an ethanol screening and a drug toxicology screening were positive in 22 and 32 patients (10% and 29%) on admission, respectively. Of the survivors, most were discharged to home with (87 [64%) or without visiting nurse supervision, and at 6 months after discharge, 16 patients (50%) in transitional care facilities were able to return to an independent level of functioning. Of the 59- to 69-year-old age group, 83 (86%) survived compared with 59 (69%) in the 70- to 79-year-old age group and 18 (47%) in the 80 years and older age group. CONCLUSIONS: In contrast to the usual male preponderance in patients with thermal injury, older women, many of whom are widowed, constituted almost half of the older patients admitted to the hospital. Modalities for injury prevention are necessary to provide optimal and safe household environments for a growing population of older persons.     

Clinical, morphologic, and cytogenetic characteristics of 26 patients with acute erythroblastic leukemia.

We have performed a retrospective analysis of the clinical, morphologic, and cytogenetic findings in 26 patients diagnosed between January 1969 and September 1991 with acute erythroblastic leukemia de novo (EL or AML-M6). Clonal chromosomal abnormalities were found in 20 (77%) patients (95% confidence interval [CI], 61% to 93%). Loss of all or part of the long arm (q) of chromosomes 5 and/or 7 was observed in 17 (65%) patients (95% CI, 47% to 83%). In addition, the karyotypes were often complex, with multiple abnormalities and subclones. Among the remaining nine patients, six had a normal karyotype and one each had trisomy 8, t(3;3), or t(3;5). The overall frequency of abnormalities of chromosomes 5 and/or 7 observed in our M6 patients is similar to that observed in our patients with therapy-related acute myeloid leukemia (t-AML; 99 of 129 patients, 77%), but substantially higher than that noted in our other patients with AML de novo (French-American-British [FAB] subtypes M1-M5: 52 of 334 patients, 16%). Our M6 patients with abnormalities of chromosomes 5 and/or 7 were older and had a shorter median survival (16 v 77 weeks [P = .005]) than did the M6 patients without these abnormalities. We found no correlation between morphologic features and either cytogenetic abnormalities or clinical outcome. Of note was the finding that the percentage of myeloblasts, which may account for only a small fraction of the total marrow elements when the revised FAB criteria are applied, had no bearing on prognosis. We conclude that acute erythroblastic leukemia, when defined by morphologic criteria, consists of two distinctive subgroups: one group tends to be older, has complex cytogenetic abnormalities, especially of chromosomes 5 and/or 7, and shares biologic and clinical features with t-AML; the other group, with simple or no detectable cytogenetic abnormalities, has a more favorable prognosis when treated with intensive chemotherapy.     

hsp70, hsp32, and grp78 are increased in thermally injured skin with and without antithrombin(human) concentrate infusion

An acquired deficiency of antithrombin (AT), an anti-inflammatory protein, develops in patients with thermal injuries. Skin thermotolerance is regulated by heat shock protein (hsp) genes. hsp70, hsp32, hsp27, and glucose-regulated protein78 (grp78) were studied in burned and unburned human skin to determine whether correction of the AT deficiency modulated the intensity of expression of these proteins. Fifty-four human skin samples were prepared by Western blot analysis: 11 unburned and 22 burned control skin samples and 7 unburned and 14 burned skin samples from patients treated with AT(Human), or AT(H). The intensity of hsp32 expression in burned AT(H)-treated skin (P < .001) and in burned control skin (P < .01) was significantly increased compared with unburned control skin. The intensity of expression of hsp70 was statistically significant in burned AT(H)-treated skin compared with unburned control skin (P < .02), as was that of grp78 (P < .01). Thermally injured skin with or without AT(H) treatment had an increased expression of hsp70, hsp32, and grp78 compared with unburned control skin.     

Granulocytic sarcoma of the ileum treated by bone marrow transplantation

An 8-year-old boy with a granulocytic sarcoma of the proximal ileum metastatic to mesenteric lymph nodes was placed into complete remission with surgical excision of the primary tumor and conventional induction chemotherapy with daunorubicin and cytosine arabinoside. He was then treated with high dose cytosine arabinoside, fractionated total body irradiation, and allogeneic marrow transplantation from his 22-month-old brother who was completely matched at the major histocompatibility complex. Methotrexate was given following the transplant to prevent graft-versus-host disease (GVHD). His post-transplantation course was complicated by a transient autoimmune hemolytic anemia related to an ABO blood group incompatibility and hepatic fungal microabscesses which responded to Amphotericin therapy. Four years following the transplant the patient remains in complete remission. The prognosis for patients with granulocytic sarcoma has been poor although, perhaps, improved over the past decade. This is the first published case report of successful treatment of a granulocytic sarcoma of the ileum by allogeneic marrow transplantation.     

Frequency of burn-trauma patients in an urban setting

BACKGROUND: Burn-trauma patient encounters constitute 5% of the emergency department population. HYPOTHESIS: A large urban hospital will treat twice as many (ie, 10%) burn-trauma patients. DESIGN: Retrospective 44-month study. SETTING: Metropolitan county hospital. PATIENTS: Population-based sample of burn-only (n = 1102), burn-trauma (n = 120), and assault burn-trauma (n = 43) patients. MAIN OUTCOME MEASURES: Frequency and demographics. RESULTS: Just under 10% (n = 120) of the burn population had burn-trauma injuries. The mean +/- SD Injury Severity Score was 12 +/- 12 in these burn-trauma patients: 4 +/- 2 in outpatients and 14 +/- 13 in inpatients. The burn-only and burn-trauma groups had similar age ranges, ethnic distribution, frequency of inhalation injury, substance abuse, malnutrition, sepsis, pneumonia, diabetes mellitus, percentage total burn surface area, number of procedures, grafted areas, and mortality. Forty-three burn-trauma patients (35.8%) sustained injuries due to assault, compared with 123 (11.2%) in the burn-only group (P<.001). Burn-trauma patients who were assaulted had a mean +/- SD Injury Severity Score of 11 +/- 10. There was a significantly increased male-female ratio among the assault burn-trauma patients (6:1) compared with the burn-trauma (3:1) and burn-only (2.3:1) groups (P<.04). Most of these injuries were caused by an unknown assailant, in connection with an automobile, a motorcycle, a bicycle, or pedestrians intentionally struck by moving vehicles, or by child abuse. The main mechanism of injury was contact in 57 burn-trauma patients (47.5%), compared with 127 (11.5%) in the burn-only group (P<.001). CONCLUSIONS: A large urban population will have an increased frequency (2-fold in our center) of burn-trauma injuries. Assault and child abuse are significant contributory factors to burn-trauma injuries in this population.     

Fractures in the under-3-year-old age cohort.

One hundred twenty-four urban children under 3 years of age admitted for fractures were retrospectively reviewed to determine the frequency of accidental and nonaccidental causes in this population. The fractures were categorized according to their mechanisms: motor-vehicle passenger or pedestrian accident, other accidents, or child abuse. There were no differences in the frequency of fractures by race, date of birth, or season in which the injury occurred. Skull fractures were most frequent (62%), followed by femur fractures (11%). There was a 26% increase in fractures between 1987 and 1989, especially in the non-motor vehicle cohort. Caretaker ignorance and/or carelessness was a common cause of fractures in the infant and toddler age group. Injuries were still occurring in spite of infant care seat use. The American public must be educated in preventive medicine and safety to decrease the senseless morbidity of our greatest resource.     

Y‐Chromosomal Lineages of Latvians in the Context of the Genetic Variation of the Eastern‐Baltic Region

Variations of the nonrecombining Y‐chromosomal region were investigated in 159 unrelated Baltic‐speaking ethnic Latvians from four different geographic regions, using 28 biallelic markers and 12 short tandem repeats. Eleven different haplogroups (hgs) were detected in a regionally homogeneous Latvian population, among which N1c, R1a, and I1 cover more than 85% of its paternal lineages. When compared its closest geographic neighbors, the composition of the Latvian Y‐chromosomal gene pool was found to be very similar to those of Lithuanians and Estonians. Despite the comparable frequency distribution of hg N1c in Latvians and Lithuanians with the Finno‐Ugric‐speaking populations from the Eastern coast of the Baltic Sea, the observed differences in allelic variances of N1c haplotypes between these two groups are in concordance with the previously stated hypothesis of different dispersal ways of this lineage in the region. More than a third of Latvian paternal lineages belong specifically to a recently defined R1a‐M558 hg, indicating an influence from a common source within Eastern Slavic populations on the formation of the present‐day Latvian Y‐chromosome gene pool.