The periodic acid-schiff reaction and prognosis in children with acute lymphoblastic leukemia

1979 ◽  
Vol 6 (1) ◽  
pp. 27-34 ◽  
Author(s):  
R. Beverly Raney ◽  
Robert S. Festa ◽  
Maria T. G. Waldman ◽  
David Manson ◽  
Hie-Won L. Hann
Blood ◽  
1986 ◽  
Vol 68 (2) ◽  
pp. 406-411 ◽  
Author(s):  
J Fradera ◽  
E Velez-Garcia ◽  
JG White

Abstract The classification of the acute leukemias depends mainly on the morphologic and cytochemical evaluation of the blast forms. One of the main accepted morphologic criteria in the differentiation between acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML) is the absence of granules in the blast cells of ALL. We evaluated a patient with ALL in whom granules were present in the cytoplasm of 35% of the blast cells, as seen in AML. Cytochemical evaluation was performed, including periodic acid-Schiff reaction, Sudan black B, alpha-naphthyl acetate, alpha-naphthyl butyrate, naphthol AS-D chloroacetate, and acid phosphatase stains. The results of these studies confirmed the morphologic impression and diagnosis of ALL. Ultrastructural evaluation revealed that the granules consisted of many tiny vesicles closely packed together in a proteinaceous matrix, resembling to some extent the inclusions described in lymphocytes in the Chediak-Higashi syndrome, but clearly different. The morphologic, cytochemical, and ultrastructural studies of this unique case are presented in detail. To our knowledge, this is the first time that such granules have been described in blast cells of ALL.


Blood ◽  
1986 ◽  
Vol 68 (2) ◽  
pp. 406-411
Author(s):  
J Fradera ◽  
E Velez-Garcia ◽  
JG White

The classification of the acute leukemias depends mainly on the morphologic and cytochemical evaluation of the blast forms. One of the main accepted morphologic criteria in the differentiation between acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML) is the absence of granules in the blast cells of ALL. We evaluated a patient with ALL in whom granules were present in the cytoplasm of 35% of the blast cells, as seen in AML. Cytochemical evaluation was performed, including periodic acid-Schiff reaction, Sudan black B, alpha-naphthyl acetate, alpha-naphthyl butyrate, naphthol AS-D chloroacetate, and acid phosphatase stains. The results of these studies confirmed the morphologic impression and diagnosis of ALL. Ultrastructural evaluation revealed that the granules consisted of many tiny vesicles closely packed together in a proteinaceous matrix, resembling to some extent the inclusions described in lymphocytes in the Chediak-Higashi syndrome, but clearly different. The morphologic, cytochemical, and ultrastructural studies of this unique case are presented in detail. To our knowledge, this is the first time that such granules have been described in blast cells of ALL.


1985 ◽  
Vol 83 (4) ◽  
pp. 494-497
Author(s):  
Gary R. Lanham ◽  
David K. Kalwinsky ◽  
Dorothy L. Williams ◽  
Susan L. Melvin ◽  
Jerry R. Harber ◽  
...  

Blood ◽  
1981 ◽  
Vol 58 (1) ◽  
pp. 14-19 ◽  
Author(s):  
GM Brodeur ◽  
DL Williams ◽  
AT Look ◽  
WP Bowman ◽  
DK Kalwinsky

We describe two adolescent girls with acute lymphoblastic leukemia (ALL) whose leukemia cells were near-haploid. Their lymphoblasts stained in a block pattern with periodic acid Schiff and had “common ALL” surface markers confirmed by indirect immunofluorescence. Each patient had two populations of blasts, one near-haploid and one hyperdiploid, which was an exact doubling of the near-haploid karyotype. The first patient had a predominant population of cells with 26 chromosomes and a few with 52, while the second had a predominance of cells with 56 and a minority with 28. Flow cytometric analysis of DNA content initially detected the minor near-haploid population in the second patient, which was confirmed later by cytogenetic review of the marrow sample. In addition to our two patients, only four patients have been reported with near-haploid ALL. Of these six, five were girls, five were adolescents, and five had short survivals (median, 10 mo). All six had disomy of chromosome 21 with or without disomy for chromosomes 10, 14, 18, or X (four patients each). Thus, near-haploid ALL may represent a unique subgroup of ALL with a poor prognosis. To detect these and other possible subgroups, we have included cytogenetic analysis and flow cytometric analysis of DNA content in our initial evaluation of patients with ALL.


Blood ◽  
1981 ◽  
Vol 58 (1) ◽  
pp. 14-19 ◽  
Author(s):  
GM Brodeur ◽  
DL Williams ◽  
AT Look ◽  
WP Bowman ◽  
DK Kalwinsky

Abstract We describe two adolescent girls with acute lymphoblastic leukemia (ALL) whose leukemia cells were near-haploid. Their lymphoblasts stained in a block pattern with periodic acid Schiff and had “common ALL” surface markers confirmed by indirect immunofluorescence. Each patient had two populations of blasts, one near-haploid and one hyperdiploid, which was an exact doubling of the near-haploid karyotype. The first patient had a predominant population of cells with 26 chromosomes and a few with 52, while the second had a predominance of cells with 56 and a minority with 28. Flow cytometric analysis of DNA content initially detected the minor near-haploid population in the second patient, which was confirmed later by cytogenetic review of the marrow sample. In addition to our two patients, only four patients have been reported with near-haploid ALL. Of these six, five were girls, five were adolescents, and five had short survivals (median, 10 mo). All six had disomy of chromosome 21 with or without disomy for chromosomes 10, 14, 18, or X (four patients each). Thus, near-haploid ALL may represent a unique subgroup of ALL with a poor prognosis. To detect these and other possible subgroups, we have included cytogenetic analysis and flow cytometric analysis of DNA content in our initial evaluation of patients with ALL.


Blood ◽  
1977 ◽  
Vol 50 (4) ◽  
pp. 671-682
Author(s):  
LW Dow ◽  
L Borella ◽  
L Sen ◽  
RJ Aur ◽  
SL George ◽  
...  

Bone marrow lymphoblasts from 109 children admitted with untreated acute lymphoblastic leukemia (ALL) were tested for spontaneous rosette formation with sheep erythrocytes. Twenty-six children (24%) had lymphoblasts that formed rosettes (E+). Of 13 initial clinical characteristics, 8 were significantly associated with E+ lymphoblasts: mediastinal enlargement (86% of patients E+), leukocyte counts over 100 X 10(9)/liter (65% E+), nodes greater than 2 cm in any diameter (65% E+), age over 5 yr (46% E+), hemoglobin over 8 g/dl (44% E+), hepatomegaly greater than 5 cm (38% E+), boys (35% E+), and lymph node enlargement outside of the cervical area (28% E+). Spleen size, initial platelet counts, and periodic acid-Schiff scores did not distinguish E+ from E- patients. Since few patients were black and few presented with central nervous system leukemia, the association of these two characteristics with E+ blasts could not be determined. A hierarchical classification scheme and a linear logistic regression model were used to define the patterns of characteristics associated with E+ lymphoblasts. The initial clinical characteristics and the poorer course of E+ patients suggest that ALL comprises at least two biologically and clinically distinct types. The E+ ALL may result from a leukemic transformation of a non-Hodgkin lymphoma.


Blood ◽  
1977 ◽  
Vol 50 (4) ◽  
pp. 671-682 ◽  
Author(s):  
LW Dow ◽  
L Borella ◽  
L Sen ◽  
RJ Aur ◽  
SL George ◽  
...  

Abstract Bone marrow lymphoblasts from 109 children admitted with untreated acute lymphoblastic leukemia (ALL) were tested for spontaneous rosette formation with sheep erythrocytes. Twenty-six children (24%) had lymphoblasts that formed rosettes (E+). Of 13 initial clinical characteristics, 8 were significantly associated with E+ lymphoblasts: mediastinal enlargement (86% of patients E+), leukocyte counts over 100 X 10(9)/liter (65% E+), nodes greater than 2 cm in any diameter (65% E+), age over 5 yr (46% E+), hemoglobin over 8 g/dl (44% E+), hepatomegaly greater than 5 cm (38% E+), boys (35% E+), and lymph node enlargement outside of the cervical area (28% E+). Spleen size, initial platelet counts, and periodic acid-Schiff scores did not distinguish E+ from E- patients. Since few patients were black and few presented with central nervous system leukemia, the association of these two characteristics with E+ blasts could not be determined. A hierarchical classification scheme and a linear logistic regression model were used to define the patterns of characteristics associated with E+ lymphoblasts. The initial clinical characteristics and the poorer course of E+ patients suggest that ALL comprises at least two biologically and clinically distinct types. The E+ ALL may result from a leukemic transformation of a non-Hodgkin lymphoma.


1990 ◽  
Vol 152 (1) ◽  
pp. 265-279
Author(s):  
A. Corsi ◽  
A. L. Granata ◽  
O. Hudlicka

Muscle performance and structure was studied in rat soleus muscle with limited blood supply in combination with chronic muscle stimulation. Blood supply to the lower leg was restricted by ligation of the common iliac artery, electrodes were implanted in the vicinity of the sciatic nerve and ankle flexors were denervated. Three days later, soleus and gastrocnemius muscles were stimulated at 4 Hz four times a day for a period of 20 min with 2 h intervals between stimulations; this procedure was continued for 4 days. Muscle performance, histochemistry and ultrastructure were studied on the eighth day after operation in these muscles and in ischaemic unstimulated muscles with denervated ankle flexors. Both were compared with control animals. Muscles with limited blood supply developed less isometric twitch tension than control muscles (peak twitch tension in ischaemic muscle was 60.3 +/− 4.8 g g-1 muscle, mean +/− S.E.M., compared to 79.7 +/− 6.9 g g-1 in control muscle; tensions after 5 min contraction were 54.5 +/− 5.5 g g-1 in ischaemic muscle compared to 70.6 +/− 6 g g-1 in controls). Stimulated muscles with limited blood supply had higher peak (85 +/− 16.6 g g-1) and final (87 +/− 12 g g-1) tensions, and also fatigued less than muscles with limited blood supply but no stimulation. Histochemical estimation of capillary density (by staining for alkaline phosphatase) and slow (SO) and fast (FOG) fibres (by myosin ATPase staining) revealed similar capillary to fibre ratios (2.5) and a similar proportion of FOG fibres (around 18%) in all muscles. The proportion of glycogen-depleted fibres (estimated from the periodic acid Schiff reaction, PAS) in muscles removed from animals 10 min after a 5 min period of isometric twitches was significantly lower in ischaemic muscles (45.1 +/− 1.9%) than in control (80.5 +/− 1.5%) or chronically stimulated ischaemic muscles (67.3 +/− 4.0%). Electron microscopy showed disorganised myofibrils with Z-line streaming in 7.48 +/− 3.04% of fibres in muscles with limited blood supply. Swollen and degenerated mitochondria, dilated sarcoplasmic reticulum and areas of disrupted sarcolemma were also observed. Stimulated ligated muscles showed a significantly lower proportion of fibres with disorganised filaments (0.65 +/− 0.32%) and other signs of damage were much less frequent. The reduced damage and improved performance of chronically stimulated slow muscle may be the result of improved microcirculation, preventing accumulation of lactate.


2018 ◽  
Vol 399 (9) ◽  
pp. 1009-1022 ◽  
Author(s):  
Shihui Guo ◽  
Peter Briza ◽  
Viktor Magdolen ◽  
Hans Brandstetter ◽  
Peter Goettig

Abstract Human kallikrein-related peptidases 3, 4, 11, and KLK2, the activator of KLK3/PSA, belong to the prostatic group of the KLKs, whose major physiological function is semen liquefaction during the fertilization process. Notably, these KLKs are upregulated in prostate cancer and are used as clinical biomarkers or have been proposed as therapeutic targets. However, this potential awaits a detailed characterization of these proteases. In order to study glycosylated prostatic KLKs resembling the natural proteases, we used Leishmania (LEXSY) and HEK293 cells for secretory expression. Both systems allowed the subsequent purification of soluble pro-KLK zymogens with correct propeptides and of the mature forms. Periodic acid-Schiff reaction, enzymatic deglycosylation assays, and mass spectrometry confirmed the glycosylation of these KLKs. Activation of glycosylated pro-KLKs 4 and 11 turned out to be most efficient by glycosylated KLK2 and KLK4, respectively. By comparing the glycosylated prostatic KLKs with their non-glycosylated counterparts from Escherichia coli, it was observed that the N-glycans stabilize the KLK proteases and change their activation profiles and their enzymatic activity to some extent. The functional role of glycosylation in prostate-specific KLKs could pave the way to a deeper understanding of their biology and to medical applications.


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