1. Queensland Health Victim Support Service
Congenital
Leukemia
A Case Presentation
Ziba Mosayebi
Professor of Neonatology
Tehran University of Medical Sciences
Children Medical Center
1396/06/24

2. Case Presentation
A female baby with a BW : 3900 and GA of 39 weeks was born through an elective C/S .
Her birth history was unremarkable.
She was admitted to a local hospital for hyperbilirubinemia at the third day of life. Lab results:
Bilirubin: 16/2.5 , Blood group (M,N : O+)
CBC; WBC: 1200 , Hb:18.1 , Plt: 89000
CRP: 3+
LFT: Nl
At day 4 she had fever and was referred to our hospital for further evaluation.
Adam transferred to an authorised mental health service as a classified patient
I’m now going to take you through a case study to illustrate how our service supports victims.
John (20) and Adam (19) were flatmates and knew each other through university friends. They had been sharing a house for 6 months with 2 other flatmates
On the night of the offence Adam entered John’s room whilst he was studying at his desk and stabbed him multiple times in the face, head and hands with a kitchen knife
2 other flatmates were home and woke when they heard John screaming. They rushed to assist John and Adam fled from the house. The police and ambulance were called and John was taken to hospital.
Adam was located by the police in a nearby park and taken to the watch house. He was charged with attempted murder and unlawful wounding.
On interview the police became concerned that Adam was mentally unwell. He appeared to be talking to himself, had rambling speech and was saying that John had the devil inside him and he needed to cut the devil out of his body. Adam was reviewed by the Court Liaison Service who felt that he was displaying symptoms of psychosis and transferred to an authorised mental health service for treatment as a classified patient.
John was admitted to hospital for treatment of his injuries.

3. Case Presentation Clinical findings at admission was:
An axillary temperature: 39°
Green-yellow discoloration of skin
Abdominal distension
Hepatomegaly (4-5cm )
Splenomegaly (2cm)
A necrotic lesion in perineum with swelling of the left labia major.
I’m now going to take you through a case study to illustrate how our service supports victims.
John (20) and Adam (19) were flatmates and knew each other through university friends. They had been sharing a house for 6 months with 2 other flatmates
On the night of the offence Adam entered John’s room whilst he was studying at his desk and stabbed him multiple times in the face, head and hands with a kitchen knife
2 other flatmates were home and woke when they heard John screaming. They rushed to assist John and Adam fled from the house. The police and ambulance were called and John was taken to hospital.
Adam was located by the police in a nearby park and taken to the watch house. He was charged with attempted murder and unlawful wounding.
On interview the police became concerned that Adam was mentally unwell. He appeared to be talking to himself, had rambling speech and was saying that John had the devil inside him and he needed to cut the devil out of his body. Adam was reviewed by the Court Liaison Service who felt that he was displaying symptoms of psychosis and transferred to an authorised mental health service for treatment as a classified patient.
John was admitted to hospital for treatment of his injuries.

4. Resources QHVSS Resources www.health.qld.gov.au/qhvss
Seeking Answers, Being Heard:
A Resource Guide for the Victims of Mentally Ill Offenders
Contact Details:
There is a lot that I haven’t been able to cover today so I’m going to leave you with our website address. On our website you’ll find a link to our resource guide which is downloadable. This provides step by step information on the forensic mental health system for victims. We also have a range of information and fact sheets about victim rights and entitlements such as patient information orders.
This is our free call 1800 number and address if you wish to contact us.
Thankyou.

5. Case Presentation
Initial diagnosis of sepsis and necrotizing fasciitis was made and she was treated by broad spectrum antibiotics( Meropenem and Vancomycin) in addition to supportive measures.
Considering some DDx such as :
Intrauterine infections
Inborn error of metabolism
Storage diseases
Malignancies (leukemia) ???
I’m now going to take you through a case study to illustrate how our service supports victims.
John (20) and Adam (19) were flatmates and knew each other through university friends. They had been sharing a house for 6 months with 2 other flatmates
On the night of the offence Adam entered John’s room whilst he was studying at his desk and stabbed him multiple times in the face, head and hands with a kitchen knife
2 other flatmates were home and woke when they heard John screaming. They rushed to assist John and Adam fled from the house. The police and ambulance were called and John was taken to hospital.
Adam was located by the police in a nearby park and taken to the watch house. He was charged with attempted murder and unlawful wounding.
On interview the police became concerned that Adam was mentally unwell. He appeared to be talking to himself, had rambling speech and was saying that John had the devil inside him and he needed to cut the devil out of his body. Adam was reviewed by the Court Liaison Service who felt that he was displaying symptoms of psychosis and transferred to an authorised mental health service for treatment as a classified patient.
John was admitted to hospital for treatment of his injuries.

6. Case Presentation Our Lab data:
CBC; WBC: 1370(N:11%, L:77%), Hb:14 , Plt: , retic:0.3%
Pt: 26, Ptt: 48
Bilirubin: 17.6/5.9
CRP: 41.9
ABG:NL
Amonia and Lactate: NL
Ultrasound confirmed HSM , brain Us was WNL and some evidence of cellulitis was present in perineal region.
Surgical consult was requested.
I’m now going to take you through a case study to illustrate how our service supports victims.
John (20) and Adam (19) were flatmates and knew each other through university friends. They had been sharing a house for 6 months with 2 other flatmates
On the night of the offence Adam entered John’s room whilst he was studying at his desk and stabbed him multiple times in the face, head and hands with a kitchen knife
2 other flatmates were home and woke when they heard John screaming. They rushed to assist John and Adam fled from the house. The police and ambulance were called and John was taken to hospital.
Adam was located by the police in a nearby park and taken to the watch house. He was charged with attempted murder and unlawful wounding.
On interview the police became concerned that Adam was mentally unwell. He appeared to be talking to himself, had rambling speech and was saying that John had the devil inside him and he needed to cut the devil out of his body. Adam was reviewed by the Court Liaison Service who felt that he was displaying symptoms of psychosis and transferred to an authorised mental health service for treatment as a classified patient.
John was admitted to hospital for treatment of his injuries.

7. Case Presentation
During the treatment course, blood and wound culture yield Pseudomonas aeruginosa .
At the third day of admission :
CBC; WBC: 4880(P:6% ,L:52%,M:32%, atypical lymph :15%), Hb:10.9 , Plt: 5000
CBC ; WBC: 11310(P:0.2% ,L:44.6%,M:25%, blasts:32%),
Hb:8 , Plt: 25000
CBC ; WBC: 18490(P:1.04% ,L:2.6%,M:4.8%,Myelocyte:1%, blasts:52%), Hb:8 , Plt: 29000
Hematologic consultation was requested , bone marrow aspiration and flowcytometry was performed.
I’m now going to take you through a case study to illustrate how our service supports victims.
John (20) and Adam (19) were flatmates and knew each other through university friends. They had been sharing a house for 6 months with 2 other flatmates
On the night of the offence Adam entered John’s room whilst he was studying at his desk and stabbed him multiple times in the face, head and hands with a kitchen knife
2 other flatmates were home and woke when they heard John screaming. They rushed to assist John and Adam fled from the house. The police and ambulance were called and John was taken to hospital.
Adam was located by the police in a nearby park and taken to the watch house. He was charged with attempted murder and unlawful wounding.
On interview the police became concerned that Adam was mentally unwell. He appeared to be talking to himself, had rambling speech and was saying that John had the devil inside him and he needed to cut the devil out of his body. Adam was reviewed by the Court Liaison Service who felt that he was displaying symptoms of psychosis and transferred to an authorised mental health service for treatment as a classified patient.
John was admitted to hospital for treatment of his injuries.

8. Case Presentation The result was AML – M4.
I’m now going to take you through a case study to illustrate how our service supports victims.
John (20) and Adam (19) were flatmates and knew each other through university friends. They had been sharing a house for 6 months with 2 other flatmates
On the night of the offence Adam entered John’s room whilst he was studying at his desk and stabbed him multiple times in the face, head and hands with a kitchen knife
2 other flatmates were home and woke when they heard John screaming. They rushed to assist John and Adam fled from the house. The police and ambulance were called and John was taken to hospital.
Adam was located by the police in a nearby park and taken to the watch house. He was charged with attempted murder and unlawful wounding.
On interview the police became concerned that Adam was mentally unwell. He appeared to be talking to himself, had rambling speech and was saying that John had the devil inside him and he needed to cut the devil out of his body. Adam was reviewed by the Court Liaison Service who felt that he was displaying symptoms of psychosis and transferred to an authorised mental health service for treatment as a classified patient.
John was admitted to hospital for treatment of his injuries.

9. Case Presentation
Skin lesion progressed from a superficial necrotic lesion to a deep soft tissue involvement in external genitalia area. Local treatment with N/S irrigation and sterile dressing continued.
I’m now going to take you through a case study to illustrate how our service supports victims.
John (20) and Adam (19) were flatmates and knew each other through university friends. They had been sharing a house for 6 months with 2 other flatmates
On the night of the offence Adam entered John’s room whilst he was studying at his desk and stabbed him multiple times in the face, head and hands with a kitchen knife
2 other flatmates were home and woke when they heard John screaming. They rushed to assist John and Adam fled from the house. The police and ambulance were called and John was taken to hospital.
Adam was located by the police in a nearby park and taken to the watch house. He was charged with attempted murder and unlawful wounding.
On interview the police became concerned that Adam was mentally unwell. He appeared to be talking to himself, had rambling speech and was saying that John had the devil inside him and he needed to cut the devil out of his body. Adam was reviewed by the Court Liaison Service who felt that he was displaying symptoms of psychosis and transferred to an authorised mental health service for treatment as a classified patient.
John was admitted to hospital for treatment of his injuries.

10. Case Presentation Parents refused chemotherapy.
She was discharged against medical advice.
Bleeding and DIC was the cause of her death a few days later in a local hospital.
I’m now going to take you through a case study to illustrate how our service supports victims.
John (20) and Adam (19) were flatmates and knew each other through university friends. They had been sharing a house for 6 months with 2 other flatmates
On the night of the offence Adam entered John’s room whilst he was studying at his desk and stabbed him multiple times in the face, head and hands with a kitchen knife
2 other flatmates were home and woke when they heard John screaming. They rushed to assist John and Adam fled from the house. The police and ambulance were called and John was taken to hospital.
Adam was located by the police in a nearby park and taken to the watch house. He was charged with attempted murder and unlawful wounding.
On interview the police became concerned that Adam was mentally unwell. He appeared to be talking to himself, had rambling speech and was saying that John had the devil inside him and he needed to cut the devil out of his body. Adam was reviewed by the Court Liaison Service who felt that he was displaying symptoms of psychosis and transferred to an authorised mental health service for treatment as a classified patient.
John was admitted to hospital for treatment of his injuries.

11. Introduction
Leukemia is the most frequent malignancy in childhood . Less than 1% of all childhood leukemia occurs in neonates.
Patients usually present with hyperleukocytosis,hepatosplenomegaly,
CNS involvement, lymphadenopathy
and skin involvement.
Leukemia cutis occurs in
25-64% of neonates with acute
leukemia (AML or ALL).
We have procedures in place with the QPS to facilitate victim referrals. Upon receiving notification that Adam had become a classified patient, we allocated a victim support coordinator, who contacted the police to obtain John’s contact details with his consent. Our coordinator contacted John once he was released from hospital and he requested that we meet with him and his parents. He had moved out of the share house and returned to live at his parent’s home following the assault.
At this appointment we were able to conduct a needs assessment and determined that there were a range of issues that John and his parents were dealing with. John was very self conscious about scars on his face and needed to have further surgery on his hands. He had temporarily deferred his studies but was concerned about his ability to return to university.
He was suffering from trauma symptoms including nightmares and difficulty sleeping and was very anxious when people came up behind him. His parents were extremely distressed and angry about what had happened and unsure of what would happen to Adam. They were particularly anxious about whether Adam would come after John again, as he knew their address and concerned about the safety of John’s 10 year old sister who lived in the family home. The family had increased financial pressures due to John’s medical expenses and the fact that he was currently unable to resume his part time job.
We referred John to the Relationships Australia Victims Counselling Service for counselling and to Legal Aid to find out about his eligibility for criminal compensation.
We also explained the process for classified patient admissions and assisted John to apply for a CPIO, as he was eligible to receive this as a direct victim. John applied for confidentiality on his application due to his safety concerns and requested that his mother be his nominee to receive information on his behalf.
Adam’s treating doctor contacted us requesting support for Adam’s mother, who was very distressed and confused about what was going to happen to Adam. The doctor advised that Adam had been diagnosed with schizophrenia and was currently still psychotic. As is our standard practice, we allocated a separate coordinator to Adam’s mum and provided her with supportive counselling and assistance in understanding the forensic mental health system and the relationships between Adam’s illness and the offence.
Adam’s charges were then referred to the Mental Health Court.

12. Fournier's gangrene (FG) is a
Introduction
Fournier's gangrene (FG) is a
fulminant form of infective necrotizing fasciitis that involves external genital
and perineal region.
We have procedures in place with the QPS to facilitate victim referrals. Upon receiving notification that Adam had become a classified patient, we allocated a victim support coordinator, who contacted the police to obtain John’s contact details with his consent. Our coordinator contacted John once he was released from hospital and he requested that we meet with him and his parents. He had moved out of the share house and returned to live at his parent’s home following the assault.
At this appointment we were able to conduct a needs assessment and determined that there were a range of issues that John and his parents were dealing with. John was very self conscious about scars on his face and needed to have further surgery on his hands. He had temporarily deferred his studies but was concerned about his ability to return to university.
He was suffering from trauma symptoms including nightmares and difficulty sleeping and was very anxious when people came up behind him. His parents were extremely distressed and angry about what had happened and unsure of what would happen to Adam. They were particularly anxious about whether Adam would come after John again, as he knew their address and concerned about the safety of John’s 10 year old sister who lived in the family home. The family had increased financial pressures due to John’s medical expenses and the fact that he was currently unable to resume his part time job.
We referred John to the Relationships Australia Victims Counselling Service for counselling and to Legal Aid to find out about his eligibility for criminal compensation.
We also explained the process for classified patient admissions and assisted John to apply for a CPIO, as he was eligible to receive this as a direct victim. John applied for confidentiality on his application due to his safety concerns and requested that his mother be his nominee to receive information on his behalf.
Adam’s treating doctor contacted us requesting support for Adam’s mother, who was very distressed and confused about what was going to happen to Adam. The doctor advised that Adam had been diagnosed with schizophrenia and was currently still psychotic. As is our standard practice, we allocated a separate coordinator to Adam’s mum and provided her with supportive counselling and assistance in understanding the forensic mental health system and the relationships between Adam’s illness and the offence.
Adam’s charges were then referred to the Mental Health Court.

13. Introduction
This aggressive and life threatening disease is more common in adults.
It is uncommon in children and very rare in neonates.
The male to female ratio is 10:1.
A high mortality rate (50%).
Following the MHC decision, John and his parents were angry and upset with the result, feeling that Adam had gotten away with it. They were particularly upset with the finding that Adam was not criminally responsible for the offence. We provided debriefing after court to help the family come to terms with the decision
We explained the restrictions of a forensic order is, the conditions that are placed on Adam and provided the family with information about the role of the MHRT in reviewing the order every 6 months. We assisted them to understand how limited community treatment works and the ongoing risk assessment and monitoring of Adam.
We supported John to make a submission to the MHRT to request that the non contact provision continue and to apply for a Forensic Patient Information Order to enable him to receive approved information about Adam’s treatment and rehabilitation. John requested confidentiality orders because of his safety concerns.
We had ongoing contact with John and his family to provide supportive counselling and information under the FPIO and assist with referrals to other services to aid in John’s recovery.
As you can appreciate this is a very condensed version of the assistance that we have provided. Our service is not time limited and we will continue to support John and his family according to their needs.

14. Introduction Predisposing factors vary with age.
Underlying disorders in affected adults are:
Diabetes mellitus,
chronic alcoholism,
malnutrition
immunosuppressive therapy
Predisposing factors in neonates are:
Prematurity
trauma,
poor hygiene
systemic infections, omphalitis
insect bite, burns
circumcision, anorectal and periurethral diseases, strangulated hernia, phimosis
hematologic malignancies
disorders of immune system, anorectal and periurethral diseases, strangulated,
malnutrition and
Prematurity, trauma, poor hygiene, systemic infections, insect bite, burns, circumcision, disorders of immune system, anorectal and periurethral diseases, strangulated hernia, phimosis, omphalitis, varicella infection, procedures in perineal region, instrumentation of urethra and hematologic malignancies
Following the MHC decision, John and his parents were angry and upset with the result, feeling that Adam had gotten away with it. They were particularly upset with the finding that Adam was not criminally responsible for the offence. We provided debriefing after court to help the family come to terms with the decision
We explained the restrictions of a forensic order is, the conditions that are placed on Adam and provided the family with information about the role of the MHRT in reviewing the order every 6 months. We assisted them to understand how limited community treatment works and the ongoing risk assessment and monitoring of Adam.
We supported John to make a submission to the MHRT to request that the non contact provision continue and to apply for a Forensic Patient Information Order to enable him to receive approved information about Adam’s treatment and rehabilitation. John requested confidentiality orders because of his safety concerns.
We had ongoing contact with John and his family to provide supportive counselling and information under the FPIO and assist with referrals to other services to aid in John’s recovery.
As you can appreciate this is a very condensed version of the assistance that we have provided. Our service is not time limited and we will continue to support John and his family according to their needs.

15. Introduction
FG usually complicate hematologic malignancies and sometimes may presents as the first sign of the disease.
From 35 cases of FG associated with hematologic malignancies about 88% of cases were complications of treatment.
In 3 cases of AML and in one case of NHL it was the first sign of the disease.
Simultaneous occurrence of FG and congenital leukemia in neonates is very rare.
Following the MHC decision, John and his parents were angry and upset with the result, feeling that Adam had gotten away with it. They were particularly upset with the finding that Adam was not criminally responsible for the offence. We provided debriefing after court to help the family come to terms with the decision
We explained the restrictions of a forensic order is, the conditions that are placed on Adam and provided the family with information about the role of the MHRT in reviewing the order every 6 months. We assisted them to understand how limited community treatment works and the ongoing risk assessment and monitoring of Adam.
We supported John to make a submission to the MHRT to request that the non contact provision continue and to apply for a Forensic Patient Information Order to enable him to receive approved information about Adam’s treatment and rehabilitation. John requested confidentiality orders because of his safety concerns.
We had ongoing contact with John and his family to provide supportive counselling and information under the FPIO and assist with referrals to other services to aid in John’s recovery.
As you can appreciate this is a very condensed version of the assistance that we have provided. Our service is not time limited and we will continue to support John and his family according to their needs.

16. Introduction
There are two types of necrotizing fasciitis depending on the responsible organisms.
Type 1 is usually polymicrobial including aerobic and anaerobic bacteria.
Type 2 is monomicrobial and often caused by group A Streptococcus alone or in combination with Staphylococcus aureus.
The usual organisms that cause gangrene Fournier in children are streptococci, staphylococci and anaerobes
Necrotizing fasciitis with Pseudomonas aeruginosa in pediatric patients affected by acute leukemia was reported
Following the MHC decision, John and his parents were angry and upset with the result, feeling that Adam had gotten away with it. They were particularly upset with the finding that Adam was not criminally responsible for the offence. We provided debriefing after court to help the family come to terms with the decision
We explained the restrictions of a forensic order is, the conditions that are placed on Adam and provided the family with information about the role of the MHRT in reviewing the order every 6 months. We assisted them to understand how limited community treatment works and the ongoing risk assessment and monitoring of Adam.
We supported John to make a submission to the MHRT to request that the non contact provision continue and to apply for a Forensic Patient Information Order to enable him to receive approved information about Adam’s treatment and rehabilitation. John requested confidentiality orders because of his safety concerns.
We had ongoing contact with John and his family to provide supportive counselling and information under the FPIO and assist with referrals to other services to aid in John’s recovery.
As you can appreciate this is a very condensed version of the assistance that we have provided. Our service is not time limited and we will continue to support John and his family according to their needs.

17. Treatment
Early diagnosis and prompt appropriate treatment is essential in FG .
Treatment includes early IV-fluid therapy, hemodynamic stabilization, broad-spectrum antibiotics and surgical debridement of necrotic tissues.
A recent study showed successful outcome by using more conservative and selective surgical methods
Despite all the medical and surgical advances in treatment the mortality remains high.
John and his parents had very little understanding of what the Mental Health Court was. Our coordinator explained that the MHC is a supreme court which determines criminal responsibility for offences, looking at soundness of mind and fitness for trial. Information about the legal requirements for an insanity defence finding and possible court outcomes were also explained.
We assisted John to have a voice in the MHC by supporting him in making a victim statement. His statement outlined his perceptions of Adam’s behaviour on the night, how the offence had affected him and his ongoing safety concerns. Due to his fear of future retaliation by Adam, John requested confidentiality on his statement and a non contact order which asked that Adam be restricted from having contact with John or his family.
We attended the Mental Health Court hearing with John and his parents.
The court found Adam of unsound mind and placed him on a forensic order. The judge considered John’s statement and approved a non contact provision which stipulated that Adam was not permitted to have contact with John, his parents or his sister.

18. Resources
Although cutaneous involvement are commonly benign and self limiting in neonates , but they may also herald serious underlying diseases and sometimes may present as the first clinical manifestations of rare neoplasms such as hematologic malignancies.
According to this case the physicians should consider leukemia in the differential diagnosis of a newborn with clinical features of sepsis and necrotizing fasciitis.
Conclusion
There is a lot that I haven’t been able to cover today so I’m going to leave you with our website address. On our website you’ll find a link to our resource guide which is downloadable. This provides step by step information on the forensic mental health system for victims. We also have a range of information and fact sheets about victim rights and entitlements such as patient information orders.
This is our free call 1800 number and address if you wish to contact us.
Thankyou.

19. Thanks for your kind attention?