scholarly journals Long-term follow-up of hemophiliacs with lymphocytopenia or thrombocytopenia

Blood ◽  
1985 ◽  
Vol 66 (6) ◽  
pp. 1317-1320
Author(s):  
ME Eyster ◽  
DA Whitehurst ◽  
PM Catalano ◽  
CW McMillan ◽  
SH Goodnight ◽  
...  

Immunologic abnormalities resembling those seen in patients with the acquired immunodeficiency syndrome (AIDS) are frequently observed in multitransfused but otherwise healthy individuals with hemophilia. To determine whether there was clinical or laboratory evidence to suggest an abnormality of immunoregulation in persons with hemophilia before the recognition of AIDS, we examined data collected by the Hemophilia Study Group from 1975 to 1979 on 1,551 patients with factor VIII deficiency. The prevalence of lymphocytopenia and thrombocytopenia in patients over 5 years of age on entry was found to be 9.3% (94/1,013) and 5.0% (26/518), respectively. These rates were significantly different from a normal population (P less than .00001 and less than .0003). No cases meeting the definition of AIDS were noted during the study. However, on follow-up in 1984 of a cohort of 79 patients with thrombocytopenia or lymphocytopenia on two or more occasions during the study, eight patients (10%) with AIDS-related abnormalities, including idiopathic thrombocytopenic purpura, non-Hodgkin's lymphoma, generalized lymphadenopathy, and oral moniliasis without obvious cause were identified. Of the 79 patients, liver disease accounted for five of the ten deaths (12.6% mortality) observed during a minimum follow-up of five years after detection of cytopenia. Only one death was attributed to bleeding in the absence of liver disease. We conclude that (a) the frequency of lymphocytopenia and thrombocytopenia was increased in multitransfused factor VIII-deficient hemophiliacs before the advent of AIDS, and (b) persistent lymphocytopenia and thrombocytopenia appear to be strongly associated with liver disease, which was the leading cause of death in a cohort of hemophiliacs followed five or more years.

Blood ◽  
1985 ◽  
Vol 66 (6) ◽  
pp. 1317-1320 ◽  
Author(s):  
ME Eyster ◽  
DA Whitehurst ◽  
PM Catalano ◽  
CW McMillan ◽  
SH Goodnight ◽  
...  

Abstract Immunologic abnormalities resembling those seen in patients with the acquired immunodeficiency syndrome (AIDS) are frequently observed in multitransfused but otherwise healthy individuals with hemophilia. To determine whether there was clinical or laboratory evidence to suggest an abnormality of immunoregulation in persons with hemophilia before the recognition of AIDS, we examined data collected by the Hemophilia Study Group from 1975 to 1979 on 1,551 patients with factor VIII deficiency. The prevalence of lymphocytopenia and thrombocytopenia in patients over 5 years of age on entry was found to be 9.3% (94/1,013) and 5.0% (26/518), respectively. These rates were significantly different from a normal population (P less than .00001 and less than .0003). No cases meeting the definition of AIDS were noted during the study. However, on follow-up in 1984 of a cohort of 79 patients with thrombocytopenia or lymphocytopenia on two or more occasions during the study, eight patients (10%) with AIDS-related abnormalities, including idiopathic thrombocytopenic purpura, non-Hodgkin's lymphoma, generalized lymphadenopathy, and oral moniliasis without obvious cause were identified. Of the 79 patients, liver disease accounted for five of the ten deaths (12.6% mortality) observed during a minimum follow-up of five years after detection of cytopenia. Only one death was attributed to bleeding in the absence of liver disease. We conclude that (a) the frequency of lymphocytopenia and thrombocytopenia was increased in multitransfused factor VIII-deficient hemophiliacs before the advent of AIDS, and (b) persistent lymphocytopenia and thrombocytopenia appear to be strongly associated with liver disease, which was the leading cause of death in a cohort of hemophiliacs followed five or more years.


1994 ◽  
Vol 4 (1) ◽  
pp. 53-58
Author(s):  
F. Specchiulli ◽  
L. Scialpi ◽  
G. Solafino ◽  
L. Battelli ◽  
L. Nitti

In CHD (Congenital Hip Dislocation), the elements which determine the degree and quality of acetabular growth are not clear. This has caused a great deal of controversy on the capability of development of the cotyloid cavity, hence on the indications to reconstructive surgical treatment. In order to study the behavior of che cotyloid cavity, two groups of patients were taken into consideration: normal subjects and subjects with CHD. In normal subjects the median value of the Hingelreiner angle was 19°–4'± 1° (normal limit), at 1 year old. The acetabular index decreases rapidly until becoming stable at adult values at the age of 8-10 years of age. The distinctive characteristics of the hip with spontaneous recovery from cotyloid dysplasia could be defined as follows: a) the higher critical value on average is reached after 24 months of treatment; b) once the borderline is reached, the dislocated hip evolves in the same way as the healthy hip; c) the earlier treatment is started, the sooner correction of the H angle is obtained; d) the cotyloid cavity continues to develop even after 5 years from reduction. In CHD with terminal residual dysplasia, an initial correction of the H angle is followed by a sudden interruption in acetabular development, which remains inadequate and will never reach normal values. These data allow not only the definition of the acetabular growth potential, but also the establishment of more precise indications for reconstructive surgical treatment.


2000 ◽  
Vol 70 (9) ◽  
pp. 1335-1342 ◽  
Author(s):  
Ashok Jain ◽  
Andrea DiMartini ◽  
Randeep Kashyap ◽  
Ada Youk ◽  
Susan Rohal ◽  
...  

2011 ◽  
Vol 37 (8) ◽  
pp. S136
Author(s):  
A. Potthoff ◽  
F. Sandkühler ◽  
B. Boozari ◽  
M.P. Manns ◽  
M.J. Gebel ◽  
...  

1984 ◽  
Vol 49 (1) ◽  
pp. 53-59 ◽  
Author(s):  
C. Miyashita ◽  
P. Hellstern ◽  
M. K�hler ◽  
G. Blohn ◽  
E. Wenzel

1998 ◽  
pp. 160-163 ◽  
Author(s):  
NC Thalassinos ◽  
S Tsagarakis ◽  
G Ioannides ◽  
I Tzavara ◽  
C Papavasiliou

Radiotherapy (RT) has long been used in the treatment of acromegaly, but confusion regarding the definition of biochemical cure has hampered interpretation of previous reports on the outcome of this treatment. In the present study we present additional data using the currently accepted criteria of biochemical cure in a large group of patients followed up by our department. Forty-six acromegalic patients were treated with external beam megavoltage RT and followed up for a mean of 7.6 years (range 2-22 years). Only four patients had had previous surgical treatment by either transsphenoidal or transfrontal routes. Following RT, mean basal GH levels decreased from 30.9 ng/ml (5-96 ng/ml) to 11.5 ng/ml (1-36 ng/ml) at 10 years of follow up with a further fall to 6.1 ng/ml (1-29 ng/ml) in those patients followed up for more than 10 years. As a result, although mean GH levels of less than 5 ng/ml were achieved in 9/28 (30.1%) at 5 years, 6/19 (31.6%) at 10 years, and in 6/11 (54.5%) of those patients followed up for more than 10 years post-RT, only 0/28 (0%), 7/28 (25%), 4/19 (21%) and 1/11 (1%) achieved GH levels of <2.5 ng/ml at 2, 5. 10 and >10 years following RT. Thus, in the whole series only 10/48 (20.8%) patients showed a decrease of GH level to less than 2.5 ng/ml at their latest follow up. Hypopituitarism as a result of RT was only infrequently observed in this series; gonadal deficiency developed in 12 (26.6%) patients, thyrotrophin (TSH) deficiency in 3 (6.6%) and adrenocorticotrophin deficiency in 2 (4.4%). In conclusion, megavoltage RT is an effective treatment for the control of GH hypersecretion in acromegaly, with a continuing lowering effect for several years following RT but seldom leads to safe GH levels.


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