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Biomedical subjects

A J Altman

Publications and source records attributed to A J Altman.

At least 19 recordsLinked to original sources

Review of the cytogenetic changes in acute megakaryoblastic leukemia: one disease or several?

The karyotypes of 116 cases of acute megakaryoblastic leukemia (AMKL) were reviewed, including 43 pediatric patients with Down syndrome (DS) and 73 non-DS patients. DS patients with AMKL often had a history of transient leukemia or myelodysplasia with an early age of onset of AMKL (median 23 months). In these patients, the frequency of additional cytogenetic change (numerical or structural) was low, with 10 of the 43 DS patients showing no additional cytogenetic change. A second group of patients had t(1;22)(p13;q13) or other cytogenetic abnormality involving 22q13. These patients had no history of transient leukemia but showed very early onset of AMKL. In this group of patients, marked organomegaly was noted; these patients also showed few specific additional cytogenetic changes. The remaining AMKL patients had a median age of 30 years with much more frequent cytogenetic changes, including rearrangement of 3q21 and 3q26-27, trisomy 21, and other specific changes. Based on the karyotype and clinical data, we hypothesize that AMKL may represent at least three separate disease entities with different genetic alterations giving rise to similar, but not identical, disorders. Subclassification of AMKL on the basis of the cytogenetic changes in the leukemic cells appears to be justified.

Adolescent↗

Scleritis and Streptococcus pneumoniae.

We retrospectively review our experience with four patients with Streptococcus pneumoniae scleritis. Two of the patients had been exposed to beta irradiation after pterygium removal 4 and 13 years previously. One patient had a 3-year history of chronic anterior nodular scleritis, and one patient had severe rheumatoid arthritis. All were treated with intensive i.v. and topical fortified antibiotics. In two of the cases, the infection was controlled and visual acuity returned to 20/30 and 20/60. In one patient, infectious scleritis progressed to endophthalmitis. This eye ultimately became phthisical and required enucleation because of chronic pain. In the remaining patient, infectious scleritis led to perforation, which required a corneal-scleral patch graft. This patient had a final visual acuity of counting fingers. An infectious etiology should be suspected in cases of necrotizing scleritis associated with a purulent discharge, and appropriate smears and cultures should be obtained. Infectious scleritis can be caused by streptococcal organisms. Appropriate topical and intravenous antibiotic treatment is effective in some cases.

Aged↗

Septicemia in pediatric oncology patients: the significance of viridans streptococcal infections.

One hundred nine consecutive episodes of septicemia were retrospectively evaluated in 61 children with malignancy. In addition, the records of all pediatric oncology patients who received high-dose cytarabine (HDAC) chemotherapy were reviewed. Gram-positive organisms accounted for 82.6% of the septicemic episodes. In the total group, coagulase-negative staphylococci and viridans streptococci accounted for 35.8% and 28.4% of the episodes, respectively. In granulocytopenic patients, viridans streptococci were the most common pathogens (36.8%). In the subset of patients who received HDAC, 62.5% of the septicemic episodes were caused by viridans streptococci. Pulmonary complications developed in nine (29%) of the total cases of viridans streptococcal sepsis, whereas these complications occurred in only eight (10.3%) of the septic episodes caused by other organisms. In patients who had viridans septicemia, prior treatment with HDAC did not increase the incidence of pulmonary complications. In septic children with malignancy, our results demonstrate a high incidence of gram-positive organisms, including viridans streptococci, which were once regarded as culture contaminants.

Adolescent↗

Clinical features and biological implications of acute mixed lineage (hybrid) leukemias.

The composite phenotype of a population of leukemic blast cells is derived through analysis of morphology, cytochemistry, cytogenetics, surface antigens, and gene structure. When analyzed in such a fashion, approximately 10-25% of childhood acute leukemias will show markers of more than one lineage; these may be coexpressed on individual cells (biphenotypic) or appear on two distinct blast populations (bilineal or biclonal). Occasionally, there is conversion from one leukemic phenotype at diagnosis to another phenotype at relapse (lineage shift). Mixed lineage features appear to have biologic and prognostic significance. Some specific mixed lineage leukemia syndromes have been identified; among them are acute nonlymphoid leukemia with T-lymphoid features, CD7+, CD4-, CD8- acute leukemia, CD2+/CD19+ acute lymphoid leukemia, and acute leukemias associated with specific cytogenetic markers, e.g., t(4;11) and t(9;22). In general, these forms of acute leukemia along with others with mixed lineage markers have a poor prognosis, and new therapeutic approaches appear to be indicated.

Antigens, Differentiation↗

Severe hyponatremia after repeated intravenous administration of desmopressin.

Desmopressin (DDAVP) has recently been found to improve hemostasis in patients with congenital or acquired disorders of coagulation and to reduce operative blood loss in patients with normal hemostasis undergoing certain surgical procedures. Despite its potent antidiuretic effect, severe hyponatremia after the intravenous administration of DDAVP is felt to be rare. We report four cases of severe hyponatremia with serious clinical sequelae occurring in patients with underlying coagulopathies who were treated prophylactically with DDAVP to improve hemostasis prior to surgical procedures. Each patient received multiple (3-22) doses of DDAVP and was given intravenous hydration with hypotonic solutions before developing clinical signs and laboratory evidence of hyponatremia. We believe that the risk of significant hyponatremia after treatment with intravenous DDAVP may be higher than is generally appreciated and that patients undergoing surgical procedures, who often receive multiple doses of DDAVP and intravenous hydration, are at particular risk for this complication. Hypotonic intravenous solutions should be avoided and serum sodium levels should be monitored frequently in those patients receiving multiple doses of DDAVP.

Adenoidectomy↗

Chronic leukemias of childhood.

Chronic leukemias account for fewer than 5 per cent of childhood hematologic malignancies. The various subtypes are chronic mylocytic leukemia (adult, juvenile, and familial), chronic myelomonocytic leukemia chronic monocytic leukemia, and chronic lymphocytic leukemia. The most common of these, adult-type chronic myelocytic leukemia, is characterized by specific cytogenetic alterations; recent advances in molecular biology are linking these genetic events to the pathophysiology and course of this fascinating neoplasm.

Child↗

The breakdown of polypropylene in the human eye: is it clinically significant?

Polypropylene intracameral sutures and intraocular lens haptics have been reported to develop cracks after varying lengths of time within the eye. A study of the degradation of a 10-0 polypropylene suture and the absence of noticeable degradation on a 4-0 polypropylene intraocular lens haptic removed five years and three months after implantation is presented. The relationship of the molecular orientation of the polypropylene suture to the clinical significance of its cracks is discussed.

Aged↗

Adriamycin and cisplatin for hepatoblastoma.

Four consecutive infants and children with hepatoblastomas were treated with a combination of Adriamycin (doxorubicin) and cisplatin. Three patients had unresectable tumors and in each there was a dramatic decrease in tumor size and serum alpha-fetoprotein (AFP) levels. The tumors of two of these patients, including one with pulmonary metastases which cleared, were rendered resectable. The third patient's tumor remained unresectable but his AFP level returned to normal following radiotherapy. All three patients are disease-free, and both without metastases are off therapy from 9 to 24 months. A fourth child received the combination as adjuvant therapy following resection of an embryonal hepatoblastoma and he remains disease-free 7 months after its discontinuation. Therapy was tolerable in all patients and its principal toxicities were myelosuppression and magnesium wasting. Adriamycin and cisplatin in combination were very effective in these patients and deserve further trials, especially in unresectable and metastatic hepatoblastomas.

Antineoplastic Combined Chemotherapy Protocols↗

Cytologic diagnosis of the acute nonlymphoid leukemias. I. Morphologic, cytochemical, and ultrastructural features.

The cytologic evaluation of a case of acute leukemia proceeds in two stages: 1) assigning the leukemia to one of two major classes, acute lymphocytic leukemia (ALL) or acute nonlymphocytic leukemia (ANLL); and 2) determining the proper subclassification of ALL or ANLL to which the case belongs. Although features such as nuclear morphology, number and nature of nucleoli, and nuclear/cytoplasmic ratio are useful criteria, the major morphologic features which distinguish ANLL cells from ALL cells are the presence of azurophilic granules and/or inclusions derived from fusion of these granules (Auer bodies). In most instances these can be visualized in the conventional blood film or bone marrow preparation. Sometimes, however, granules and Auer bodies are too small or too few to be seen with the light microscope. In such cases histochemical or ultrastructural studies will aid in proper classification.

Acute Disease↗

Cytologic diagnosis of the acute nonlymphoid leukemias. II. Flow cytometry, surface markers, cytogenetics, and use of cell culture techniques.

Part I of this two-part article discussed the use of morphologic, histochemical, and ultrastructural studies for the diagnosis and classification of ANLL variants of acute nonlymphoid leukemia. However, a small proportion of acute leukemias are not amenable to definition by these techniques and have, in the past, been classified as acute undifferentiated leukemias. The use of supplemental techniques such as flow cytometry, surface marker analysis, cytogenetics, and in vitro growth patterns will often identify the correct cellular lineage for these cases.

Acute Disease↗

Acute pancreatitis in association with cytosine arabinoside therapy.

This paper reports the association of acute pancreatitis coincident with cytosine arabinoside (Ara-c) therapy in a single patient on at least two occasions. The patient had previously received L-asparaginase, but the last dose had been given 4 months prior to the onset of pancreatitis. A literature review provided two more cases of pancreatitis associated with Ara-c therapy in patients previously treated with L-asparaginase. In view of th extreme rarity of pancreatitis in patients receiving Ara-c, the possibility arises that prior treatment with L-asparaginase may predispose the pancreas to this complication.

Acute Disease↗

Giant granules and rod-shaped inclusions arising de novo in monocytes and macrophages cultured from a patient with acute monocytic leukemia.

In this paper we describe the de novo appearance of azurophilic giant granules and rod-shaped inclusions in monocytes and macrophages grown from the peripheral blood and bone marrow of a patient with acute monocytic leukemia; no such inclusions were evident in direct smears of the patient's peripheral blood or bone marrow. The cytochemical profile of the inclusions, their origin in mononuclear phagocytes only, and their development exclusively in vitro suggest that they are distinct from Auer rods, Chediak-Higashi-like giant granules, and other abnormal azurophilic inclusions previously described in patients with acute non-lymphocytic leukemia.

Adolescent↗