http://sickle.bwh.harvard.edu/aplastic_crisis.html
For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America
Showing posts with label Fifth's Disease. Show all posts
Showing posts with label Fifth's Disease. Show all posts
Thursday, July 3, 2008
Wednesday, February 13, 2008
Parvo B19 Infection Reminiscent of Myelodysplastic Syndrome in 3 Children.....
PARVOVIRUS B19 INFECTION REMINISCENT OF MYELODYSPLASTIC SYNDROME IN THREE CHILDREN WITH CHRONIC HEMOLYTIC ANEMIA
Authors: Nee Yaral a; Feride Duru a; Tansu Sipahi a; Abdurrahman Kara a; Tahsin Teziç a
Affiliation: a Department or Pediatric Hematology, Dr. Sami Ulus Children's Disease Centre, Ankara, Turkey.
Published in: Pediatric Hematology and Oncology, Volume 17, Issue 6 September 2000 , pages 475 - 482
Subjects: Oncology: Hematologic Oncology; Hematology: Hematologic Oncology; Pediatrics & Child Health;
Abstract
The authors have seen transient pancytopenia with erythroid hypoplasia and striking trilineage myelodysplasia reminiscent of true myelodysplastic syndrome (MDS) in 3 children, 1 with thalassemia intermedia and the other 2 with previously undiagnosed hereditary spherocytosis. In these 3 children transient pancytopenia and myelodysplasia coincided with serological evidence of acute parvovirus B19 (PV-B19) infection, strongly suggesting their relevance. It is of interest that these 3 cases were encountered within a period of 6 months. This might be an incidental event, but it might also be concluded that acute PV-B19 infection associated transient pancytopenia with morphological appearance of MDS may occur more frequently than reported in the literature. So, PV-B19-associated nonclonal MDS should be considered in the differential diagnosis of trueclonal MDS.
Keywords: Mds; Pancytopenia; Parvovirus B19
Authors: Nee Yaral a; Feride Duru a; Tansu Sipahi a; Abdurrahman Kara a; Tahsin Teziç a
Affiliation: a Department or Pediatric Hematology, Dr. Sami Ulus Children's Disease Centre, Ankara, Turkey.
Published in: Pediatric Hematology and Oncology, Volume 17, Issue 6 September 2000 , pages 475 - 482
Subjects: Oncology: Hematologic Oncology; Hematology: Hematologic Oncology; Pediatrics & Child Health;
Abstract
The authors have seen transient pancytopenia with erythroid hypoplasia and striking trilineage myelodysplasia reminiscent of true myelodysplastic syndrome (MDS) in 3 children, 1 with thalassemia intermedia and the other 2 with previously undiagnosed hereditary spherocytosis. In these 3 children transient pancytopenia and myelodysplasia coincided with serological evidence of acute parvovirus B19 (PV-B19) infection, strongly suggesting their relevance. It is of interest that these 3 cases were encountered within a period of 6 months. This might be an incidental event, but it might also be concluded that acute PV-B19 infection associated transient pancytopenia with morphological appearance of MDS may occur more frequently than reported in the literature. So, PV-B19-associated nonclonal MDS should be considered in the differential diagnosis of trueclonal MDS.
Keywords: Mds; Pancytopenia; Parvovirus B19
Parvo B19 - Fifth's Disease
http://www.stanford.edu/group/virus/parvo/2000/B19.html
Human Parvovirus B19
Common appearance of "slapped cheek" disease, a.k.a. fifth disease.
____
General Information
The B19 parvovirus, a member of the genus, Erythrovirus, is the
cause of
a range of clinical syndromes, the most common of which is erythema
infectiosum, or "fifth disease." This manifestation of B19 is a mild
condition seen in children and adults, and about 50% of adults are
seropositive after adolescence. Other clinical syndromes caused by B19
include Arthritis (especially in young women), Aplastic crisis in
chronic
hemolytic anemia, Chronic anemia in immunodeficiency syndromes, and
Hydrops
foetalis in newborn infants.
_____
Epidemiology
Parvovirus B19 is a very common, ubiquitous disease that tends to
produce spring epidemics in children 4-10 years of age. It is easily
transmitted through respiratory secretions and close contact, and
transmission is common in daycare centers and schools. The virus is
transmitted from person to person during the incubation period,
before a
clinical rash actually appears. By the time the rash actually does
appear,
the infected individual is no longer capable of infecting others.
Over 50%
of adults are seropositive and immune to B19.
Transplacental transmission is also possible, but unlikely. If
fetal
infection does occur, it is usually harmless. In less than 5% of all
cases,
however, primary maternal infections can lead to Hydrops foetalis,
and in
rare cases, fetal death. Transmission through blood transfusion is
also
possible.
_____
Incubation
B19 can present as a biphasic illness, with the first symptoms of
malaise occurring 8-11 days after infection and the rash appearing
between
days 17-24.
_____
Pathogenesis
Viremia reaches its peak one week after infection, at which point
the
virus is shed from the throat. Right after viremia ends, levels of
specific
IgM antibodies peak. Parvovirus B19 is selective for erythrocyte
progenitors, infecting them and inhibiting their development into
mature
erythrocytes.
The schematic representation below shows the progression of the
virologic,
hematologic and clinical events in parvovirus B19 infection:
From A.J. Zuckerman, et al., "Principles and Practice of Clinical
Virology,"
2nd Ed. Wiley, Chicheste, 1990.)
_____
Symptomology and Outcome
In the case of fifth disease, or erythema infectiosum, a "slapped-
cheek"
rash appears on the face of the child, giving him/her a flushed look.
The
rash also involves the limbs and trunk, where it takes on a lacy
appearance.
Adults may also experience joint swelling and pain that usually
resolves
fairly rapidly, but may remain for weeks. The regular rash usually
resolves
in about a week, and the individual gains immunity to future
infection.
In the case of Transient Aplastic Crisis, the patient experiences
lethargy, pallor and weakness that are all characteristic of severe
anemia.
This condition occurs as a complication in individuals with various
forms of
chronolytic anemia. Recovery usually occurs within a week, but the
condition
may be life threatening and blood transfusions may be required to
prevent
death.
Chronic anemia may be another complication of B19 infection and
has been
observed in immunodeficient patients.
Hydrops foetalis is a rare condition that is presumed to result
from
severe anemia and congestive heart failure in the fetus of PVB19
infected
mothers.
_____
Prevention and Management
There is no vaccine available against B19 and usually no
treatment is
necessary. Aplastic crisis and hemolytic anemia require supportive
care and
blood transfusion. Intravenous administration of immunoglobulin is
beneficial in the treatment of severe persistent anemia in
immunocompromised
individuals.
Washing hands frequently is probably the best method for
prevention.
Human Parvovirus B19
Common appearance of "slapped cheek" disease, a.k.a. fifth disease.
____
General Information
The B19 parvovirus, a member of the genus, Erythrovirus, is the
cause of
a range of clinical syndromes, the most common of which is erythema
infectiosum, or "fifth disease." This manifestation of B19 is a mild
condition seen in children and adults, and about 50% of adults are
seropositive after adolescence. Other clinical syndromes caused by B19
include Arthritis (especially in young women), Aplastic crisis in
chronic
hemolytic anemia, Chronic anemia in immunodeficiency syndromes, and
Hydrops
foetalis in newborn infants.
_____
Epidemiology
Parvovirus B19 is a very common, ubiquitous disease that tends to
produce spring epidemics in children 4-10 years of age. It is easily
transmitted through respiratory secretions and close contact, and
transmission is common in daycare centers and schools. The virus is
transmitted from person to person during the incubation period,
before a
clinical rash actually appears. By the time the rash actually does
appear,
the infected individual is no longer capable of infecting others.
Over 50%
of adults are seropositive and immune to B19.
Transplacental transmission is also possible, but unlikely. If
fetal
infection does occur, it is usually harmless. In less than 5% of all
cases,
however, primary maternal infections can lead to Hydrops foetalis,
and in
rare cases, fetal death. Transmission through blood transfusion is
also
possible.
_____
Incubation
B19 can present as a biphasic illness, with the first symptoms of
malaise occurring 8-11 days after infection and the rash appearing
between
days 17-24.
_____
Pathogenesis
Viremia reaches its peak one week after infection, at which point
the
virus is shed from the throat. Right after viremia ends, levels of
specific
IgM antibodies peak. Parvovirus B19 is selective for erythrocyte
progenitors, infecting them and inhibiting their development into
mature
erythrocytes.
The schematic representation below shows the progression of the
virologic,
hematologic and clinical events in parvovirus B19 infection:
From A.J. Zuckerman, et al., "Principles and Practice of Clinical
Virology,"
2nd Ed. Wiley, Chicheste, 1990.)
_____
Symptomology and Outcome
In the case of fifth disease, or erythema infectiosum, a "slapped-
cheek"
rash appears on the face of the child, giving him/her a flushed look.
The
rash also involves the limbs and trunk, where it takes on a lacy
appearance.
Adults may also experience joint swelling and pain that usually
resolves
fairly rapidly, but may remain for weeks. The regular rash usually
resolves
in about a week, and the individual gains immunity to future
infection.
In the case of Transient Aplastic Crisis, the patient experiences
lethargy, pallor and weakness that are all characteristic of severe
anemia.
This condition occurs as a complication in individuals with various
forms of
chronolytic anemia. Recovery usually occurs within a week, but the
condition
may be life threatening and blood transfusions may be required to
prevent
death.
Chronic anemia may be another complication of B19 infection and
has been
observed in immunodeficient patients.
Hydrops foetalis is a rare condition that is presumed to result
from
severe anemia and congestive heart failure in the fetus of PVB19
infected
mothers.
_____
Prevention and Management
There is no vaccine available against B19 and usually no
treatment is
necessary. Aplastic crisis and hemolytic anemia require supportive
care and
blood transfusion. Intravenous administration of immunoglobulin is
beneficial in the treatment of severe persistent anemia in
immunocompromised
individuals.
Washing hands frequently is probably the best method for
prevention.
Parvoviruses and Bone Marrow Failure
This topic comes up a lot on our email support list. Parvo B19, commonly known as Fifth's disease, can be dangerous in SDS kids. The abstract is below, the full text is located here: http://stemcells.alphamedpress.org/cgi/content/full/14/2/151
Parvoviruses and Bone Marrow Failure
Kevin E. Brown, Neal S. Young
Hematology Branch, National Heart, Lung and Blood Institute, Bethesda, Maryland, USA
Abstract
Parvovirus B19, the only known human pathogenic parvovirus, is highly tropic to human bone marrow and replicates only in erythroid progenitor cells. The basis of this erythroid tropism is the tissue distribution of the B19 cellular receptor, globoside (blood group P antigen). In individuals with underlying hemolytic disorders, infection with parvovirus B19 is the primary cause of transient aplastic crisis. In immunocompromised patients, persistent B19 infection may develop that manifests as pure red cell aplasia and chronic anemia. B19 infection in utero can result in fetal death, hydrops fetalis or congenital anemia. Diagnosis is based on examination of the bone marrow and B19 virological studies. Treatment of persistent infection with immunoglobulin leads to a rapid, marked resolution of the anemia.
Parvoviruses and Bone Marrow Failure
Kevin E. Brown, Neal S. Young
Hematology Branch, National Heart, Lung and Blood Institute, Bethesda, Maryland, USA
Abstract
Parvovirus B19, the only known human pathogenic parvovirus, is highly tropic to human bone marrow and replicates only in erythroid progenitor cells. The basis of this erythroid tropism is the tissue distribution of the B19 cellular receptor, globoside (blood group P antigen). In individuals with underlying hemolytic disorders, infection with parvovirus B19 is the primary cause of transient aplastic crisis. In immunocompromised patients, persistent B19 infection may develop that manifests as pure red cell aplasia and chronic anemia. B19 infection in utero can result in fetal death, hydrops fetalis or congenital anemia. Diagnosis is based on examination of the bone marrow and B19 virological studies. Treatment of persistent infection with immunoglobulin leads to a rapid, marked resolution of the anemia.
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