These were from a talk by Dr. S..... she gave handouts later. She said the studies are limited.
Evaluation of low blood counts
r/o other treatable causes of low blood counts
anemia-- blood loss, antibodies, erythropoietin levels
neutropenia - infections or medication
G-CSF increases neutrophils and not all patients respond. Indications for using G-CSF:
neutropenia with persistent or serious bacterial infections or fungal infections
neutropenia w/ history of recurrent bacterial/fungal infections, gingivitis or mouth sores
some centers recommend G-CSF prophylactically for persistently low neutrophil counts (<200-500)
need to weigh potential risks
G-CSF potential side effects:
bone pain
enlarged spleen (associated with chronic use)
?osteopenia (seeing it in SCN patients-not sure if it is the underlying disease or from G-CSF)
Anectdotally- kidney problems
Bone marrow exam with cytogenetics should be done prior to initiating therapy with G-CSF. No causal relationship between cytokine therapy and leukemia has been demonstrated to date, but can;t rule it out, either.
Leukemia patients have used G-CSF to get through chemotherapy and have not seen an adverse effect. Not sure if you can extrapolate this to SDS population.
Supportive care for Anemia
Transfusion
Indications for transfusion: symptomatic anemia, fatigue, exercise intolerance, rapid heart rate and breath rate, poor growth
Rough guide: transfuse when hemoglobin is <8 -- this varies from patient to patient.
Risks of transfusion
allosensitizatization: patient develops antibodies against transfused red cells or platelets such that transfused cells are rapidly destroyed (makes transplant harder)
iron overload secondary to red cell transfusion (also makes transplant more difficult) (can use chelating agents)
transfusion reaction
infection (blood borne)
high body iron stores puts you at risk for transplant complications.
supportive care for thrombocytopenia
Indications for transfusion: symptomatic, bleeding, bruising and prophylaxis prior to surgery
20,000 is usually uses, but studies show 5,000-10,000 can be okay. Must consider the patient....for instance, a toddler who is always bonking his head.....transfusion would possibly be considered at a different number.
must also check Vit K levels....low vit K levels with low platelets can cause worse problems. Also, liver function can be a cause of bleeding problems.
Risks of platelet transfusions-- infections
For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America
Showing posts with label hematology. Show all posts
Showing posts with label hematology. Show all posts
Tuesday, July 29, 2008
Camp Notes: Hematology
The Hematology session (formerly called Hematology 101)
Some of the talks gave handouts—this was one of them….so I only have a few notes. Once I get home, I will try to add more to this.
Must interpret cellularity in context with the CBC
If the CBC is normal and there is low cellularity, it may be a sampling error
Young children should be quite cellular
Be sure that the marrow is read by those who work with children’s bone marrows…..
There was talk of clones, etc……. can’t draw pictures in email! LOL
CGH is a new genetics test like FISH that paints chromosomes various colors. We were told it is in the research stages—but know one person who has had it done.
MDS/Myelodysplastic syndrome is a highly contested area in IBMFS
People with IBMFS have abnormal marrows and may look like MDS marrows. MDS diagnosis is more complicated in SDS. Adult hem/oncs would say definite MDS…but in SDS it could be normal.
Hypo cellular marrow—mild decrease—wait and watch. The functional test is the blood counts. Over time, if cellularity is dropping and if counts also drop……it is premature to go to transplant with just low cellularity and normal counts.
Notes from Hematology break o0ut session:
I asked about kids without mutations that have been called SDS-like….when looking at the marrow, can you tell they have an inherited bone marrow failure syndrome? Could normal kids have some abnormalities in their marrow.
Yes, normal kids could have something pop up, and that is why repeat marrows are done. i..e if you have an abnormality (mild) in one our of five marrows, then it would be okay for a normal person to have it—but when you have a child who has abnormalities in every marrow, it is a clue to diagnosis.
He explained that of the kids with inherited marrow failure syndromes, 50% are diagnosed…. The other 50% don’t fall into the *named* IBMFS….but they know they have a BMFS because more than one child/person in the family has it
It can be autosomal recessive like SDS or autosomal dominant like SCN or sex linked recessive like DC (only males & skips a generation)
Looking at the marrow, you may not be able to tell that a person has an IBMFS…..
If you have one cell out of 20 with a chromosomal abnormality, it can be considered to be normal. If you have 2 out of twenty, it is a clone. FISH 4% of monosomy 7 is *okay* (tried to paraphrase what he was saying. It is not normal to have 4%, but continued follow up is needed. He went on to explain that sometimes cells can be together and sludge, etc…so that is why follow up is needed.
We talked about iron stores…and there are types of iron stores that you don’t want—especially ringed sideroblasts.
CBC is a late spot for leukemia or MDS. Cannot use CBC in BMFS—must do bone marrow biopsies.
Abnormal clones—even with normal counts must be followed closely with more frequent marrows.
For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America
Some of the talks gave handouts—this was one of them….so I only have a few notes. Once I get home, I will try to add more to this.
Must interpret cellularity in context with the CBC
If the CBC is normal and there is low cellularity, it may be a sampling error
Young children should be quite cellular
Be sure that the marrow is read by those who work with children’s bone marrows…..
There was talk of clones, etc……. can’t draw pictures in email! LOL
CGH is a new genetics test like FISH that paints chromosomes various colors. We were told it is in the research stages—but know one person who has had it done.
MDS/Myelodysplastic syndrome is a highly contested area in IBMFS
People with IBMFS have abnormal marrows and may look like MDS marrows. MDS diagnosis is more complicated in SDS. Adult hem/oncs would say definite MDS…but in SDS it could be normal.
Hypo cellular marrow—mild decrease—wait and watch. The functional test is the blood counts. Over time, if cellularity is dropping and if counts also drop……it is premature to go to transplant with just low cellularity and normal counts.
Notes from Hematology break o0ut session:
I asked about kids without mutations that have been called SDS-like….when looking at the marrow, can you tell they have an inherited bone marrow failure syndrome? Could normal kids have some abnormalities in their marrow.
Yes, normal kids could have something pop up, and that is why repeat marrows are done. i..e if you have an abnormality (mild) in one our of five marrows, then it would be okay for a normal person to have it—but when you have a child who has abnormalities in every marrow, it is a clue to diagnosis.
He explained that of the kids with inherited marrow failure syndromes, 50% are diagnosed…. The other 50% don’t fall into the *named* IBMFS….but they know they have a BMFS because more than one child/person in the family has it
It can be autosomal recessive like SDS or autosomal dominant like SCN or sex linked recessive like DC (only males & skips a generation)
Looking at the marrow, you may not be able to tell that a person has an IBMFS…..
If you have one cell out of 20 with a chromosomal abnormality, it can be considered to be normal. If you have 2 out of twenty, it is a clone. FISH 4% of monosomy 7 is *okay* (tried to paraphrase what he was saying. It is not normal to have 4%, but continued follow up is needed. He went on to explain that sometimes cells can be together and sludge, etc…so that is why follow up is needed.
We talked about iron stores…and there are types of iron stores that you don’t want—especially ringed sideroblasts.
CBC is a late spot for leukemia or MDS. Cannot use CBC in BMFS—must do bone marrow biopsies.
Abnormal clones—even with normal counts must be followed closely with more frequent marrows.
For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America
Thursday, June 19, 2008
New Booklet Available
Shwachman-Diamond America is pleased to announce that we have a new booklet available. The title is Management of the Hematologic Abnormalities of Shwachman-Diamond Syndrome by Richard Harris, M.D. This booklet is excellent for parents, pediatricians and specialists. If you would like a copy (or copies) of the booklet sent to you, please send your mailing address to shwachmandiamondamerica@embarqmail.com These booklets are FREE to anyone who requests them. Visit the webstie above to request your free booklet.
The booklet includes sections on the following topics:
Description
History
Diagnosis
Management of Neutropenia
Management of Fevers in SDS patients
Antibiotic coverage prior to Dental Work or Surgical Procedures
Management of other Cytopenias
Management of Anemia during Surgical Procedures
Management of MDS
Management of Leukemia
Bone Marrow Transplantation for SDS
And also includes the following:
K-M Survival Plots
Important Contacts
References
Glossary
All of us at Shwachman-Diamond America would like to take this opportunity to thank Dr. Harris for all of his hard work and dedication to this project. Thank you, Dr. Harris, from the bottom of our hearts! Your dedication to all of our SDS and SDS-like children is appreciated more than you will ever know.
For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America
The booklet includes sections on the following topics:
Description
History
Diagnosis
Management of Neutropenia
Management of Fevers in SDS patients
Antibiotic coverage prior to Dental Work or Surgical Procedures
Management of other Cytopenias
Management of Anemia during Surgical Procedures
Management of MDS
Management of Leukemia
Bone Marrow Transplantation for SDS
And also includes the following:
K-M Survival Plots
Important Contacts
References
Glossary
All of us at Shwachman-Diamond America would like to take this opportunity to thank Dr. Harris for all of his hard work and dedication to this project. Thank you, Dr. Harris, from the bottom of our hearts! Your dedication to all of our SDS and SDS-like children is appreciated more than you will ever know.
For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America
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