SURVEY OF UK HAEMATOLOGY EXPERIENCE IN MASTOCYOSIS

Survey of UK Haematology Experience in Mastocytosis

Systemic mastocytosis is a rare haematological neoplasm. In lieu of a registry, this survey is being conducted on behalf of Guy’s and St Thomas’ NHS Foundation Trust and the NCRI MPN sub-group to collate information on the current experience of haematologists managing patients with systemic mastocytosis. The aim is to examine the current practice and numbers of patients being seen within the UK to help inform any discussions for approvals of up and coming targeted drugs.

This survey should take between 10-15 minutes to complete and we would be grateful if your answers could be submitted by Friday 1st October. If you have any queries, please do not hesitate to contact us via the details below. Many thanks in advance for your help with this.

Dr Deepti Radia

Consultant Haematologist at Guy's and St Thomas' Hospital

Email: deepti.radia@nhs.net

Dr Priya Sriskandarajah

Clinical Fellow at Guy's and St Thomas' Hospital

Email: priya.sriskandarajah@nhs.net

Question Title

* 1. Where is your hospital based?

North East England

North West England

South East England

South West England

Greater London

Scotland

Wales

Northern Ireland

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* 2. Do you see Systemic Mastocytosis patients in your practice?

Yes

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* 3. If so, how many? Please select one of the options below and specify the number of patients you have seen in the comment box.

1-5

6-10

11-15

16-20

20+

Other (please specify)

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* 4. Which department were they referred from? Please select as many as appropriate

Immunology

Allergy

Dermatology

Gastroenterology

Respiratory

Haematology

Other (please specify)

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* 5. What type of systemic mastocytosis patients have you managed in your practice?

	1-5	6-10	11-15	16-20	20+
Cutaneous Mastocytosis/MCIS	Cutaneous Mastocytosis/MCIS 1-5	Cutaneous Mastocytosis/MCIS 6-10	Cutaneous Mastocytosis/MCIS 11-15	Cutaneous Mastocytosis/MCIS 16-20	Cutaneous Mastocytosis/MCIS 20+
Bone marrow mastocytosis	Bone marrow mastocytosis 1-5	Bone marrow mastocytosis 6-10	Bone marrow mastocytosis 11-15	Bone marrow mastocytosis 16-20	Bone marrow mastocytosis 20+
Indolent Systemic Mastocytosis	Indolent Systemic Mastocytosis 1-5	Indolent Systemic Mastocytosis 6-10	Indolent Systemic Mastocytosis 11-15	Indolent Systemic Mastocytosis 16-20	Indolent Systemic Mastocytosis 20+
Smouldering Systemic Mastocytosis	Smouldering Systemic Mastocytosis 1-5	Smouldering Systemic Mastocytosis 6-10	Smouldering Systemic Mastocytosis 11-15	Smouldering Systemic Mastocytosis 16-20	Smouldering Systemic Mastocytosis 20+
Systemic Mastocytosis with associated haematological neoplasm	Systemic Mastocytosis with associated haematological neoplasm 1-5	Systemic Mastocytosis with associated haematological neoplasm 6-10	Systemic Mastocytosis with associated haematological neoplasm 11-15	Systemic Mastocytosis with associated haematological neoplasm 16-20	Systemic Mastocytosis with associated haematological neoplasm 20+
Aggressive systemic mastocytosis	Aggressive systemic mastocytosis 1-5	Aggressive systemic mastocytosis 6-10	Aggressive systemic mastocytosis 11-15	Aggressive systemic mastocytosis 16-20	Aggressive systemic mastocytosis 20+
Mast cell leukaemia	Mast cell leukaemia 1-5	Mast cell leukaemia 6-10	Mast cell leukaemia 11-15	Mast cell leukaemia 16-20	Mast cell leukaemia 20+

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* 6. If you have seen SM patients with an associated haematological neoplasm, please select the type of AHN from below.

	1-2	3-4	5-6	7-8	9-10
CMML	CMML 1-2	CMML 3-4	CMML 5-6	CMML 7-8	CMML 9-10
MDS	MDS 1-2	MDS 3-4	MDS 5-6	MDS 7-8	MDS 9-10
MDS/MPN	MDS/MPN 1-2	MDS/MPN 3-4	MDS/MPN 5-6	MDS/MPN 7-8	MDS/MPN 9-10
MPN	MPN 1-2	MPN 3-4	MPN 5-6	MPN 7-8	MPN 9-10
MF	MF 1-2	MF 3-4	MF 5-6	MF 7-8	MF 9-10
PV	PV 1-2	PV 3-4	PV 5-6	PV 7-8	PV 9-10
ET	ET 1-2	ET 3-4	ET 5-6	ET 7-8	ET 9-10
AML	AML 1-2	AML 3-4	AML 5-6	AML 7-8	AML 9-10
Lymphoid	Lymphoid 1-2	Lymphoid 3-4	Lymphoid 5-6	Lymphoid 7-8	Lymphoid 9-10
Plasma cell dyscrasia	Plasma cell dyscrasia 1-2	Plasma cell dyscrasia 3-4	Plasma cell dyscrasia 5-6	Plasma cell dyscrasia 7-8	Plasma cell dyscrasia 9-10

Other (please specify)

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* 7. Please can you detail the gender distribution of patients you have managed in your practice.

	1-5	6-10	11-15	16-20	20+
Female	Female 1-5	Female 6-10	Female 11-15	Female 16-20	Female 20+
Male	Male 1-5	Male 6-10	Male 11-15	Male 16-20	Male 20+

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* 8. Please select the age distribution of SM patients you have seen in your practise.

	1-5	6-10	11-15	16-20	20+
18-25 yrs	18-25 yrs 1-5	18-25 yrs 6-10	18-25 yrs 11-15	18-25 yrs 16-20	18-25 yrs 20+
26-30 yrs	26-30 yrs 1-5	26-30 yrs 6-10	26-30 yrs 11-15	26-30 yrs 16-20	26-30 yrs 20+
31-40 yrs	31-40 yrs 1-5	31-40 yrs 6-10	31-40 yrs 11-15	31-40 yrs 16-20	31-40 yrs 20+
41-50 yrs	41-50 yrs 1-5	41-50 yrs 6-10	41-50 yrs 11-15	41-50 yrs 16-20	41-50 yrs 20+
51-60 yrs	51-60 yrs 1-5	51-60 yrs 6-10	51-60 yrs 11-15	51-60 yrs 16-20	51-60 yrs 20+
61-70 yrs	61-70 yrs 1-5	61-70 yrs 6-10	61-70 yrs 11-15	61-70 yrs 16-20	61-70 yrs 20+
75+ yrs	75+ yrs 1-5	75+ yrs 6-10	75+ yrs 11-15	75+ yrs 16-20	75+ yrs 20+

Question Title

* 9. For those with advanced SM (i.e. ASM; SM-AHN; MCL), which additional mutations did you also identify?

c-KIT D816V

c-KIT alternate mutation

SRSF2

AXL1

RUNX1

JAK2

CALR

Other (please specify)

Question Title

* 10. Of those patients you have seen, how many have received the following symptomatic/supportive treatment? Please select as many as applicable.

	1-5	6-10	11-15	16-20	20+
Anti-mediators	Anti-mediators 1-5	Anti-mediators 6-10	Anti-mediators 11-15	Anti-mediators 16-20	Anti-mediators 20+
Steroids	Steroids 1-5	Steroids 6-10	Steroids 11-15	Steroids 16-20	Steroids 20+
Bisphosphonates	Bisphosphonates 1-5	Bisphosphonates 6-10	Bisphosphonates 11-15	Bisphosphonates 16-20	Bisphosphonates 20+
Immune tolerance therapy (ITT)	Immune tolerance therapy (ITT) 1-5	Immune tolerance therapy (ITT) 6-10	Immune tolerance therapy (ITT) 11-15	Immune tolerance therapy (ITT) 16-20	Immune tolerance therapy (ITT) 20+
Omalizumab	Omalizumab 1-5	Omalizumab 6-10	Omalizumab 11-15	Omalizumab 16-20	Omalizumab 20+

Other (please specify)

Question Title

* 11. How many have received any of the following cytoreductive therapies?

	1-5	6-10	11-15	16-20	20+
Steroids	Steroids 1-5	Steroids 6-10	Steroids 11-15	Steroids 16-20	Steroids 20+
Interferon/Pegylated Interferon	Interferon/Pegylated Interferon 1-5	Interferon/Pegylated Interferon 6-10	Interferon/Pegylated Interferon 11-15	Interferon/Pegylated Interferon 16-20	Interferon/Pegylated Interferon 20+
Cladribine	Cladribine 1-5	Cladribine 6-10	Cladribine 11-15	Cladribine 16-20	Cladribine 20+
Hydroxycarbamide	Hydroxycarbamide 1-5	Hydroxycarbamide 6-10	Hydroxycarbamide 11-15	Hydroxycarbamide 16-20	Hydroxycarbamide 20+
Azacitidine	Azacitidine 1-5	Azacitidine 6-10	Azacitidine 11-15	Azacitidine 16-20	Azacitidine 20+
Midostaurin (Trial/Compassionate access)	Midostaurin (Trial/Compassionate access) 1-5	Midostaurin (Trial/Compassionate access) 6-10	Midostaurin (Trial/Compassionate access) 11-15	Midostaurin (Trial/Compassionate access) 16-20	Midostaurin (Trial/Compassionate access) 20+
Avapritinib (Trial/Compassionate access)	Avapritinib (Trial/Compassionate access) 1-5	Avapritinib (Trial/Compassionate access) 6-10	Avapritinib (Trial/Compassionate access) 11-15	Avapritinib (Trial/Compassionate access) 16-20	Avapritinib (Trial/Compassionate access) 20+
Ruxolitinib	Ruxolitinib 1-5	Ruxolitinib 6-10	Ruxolitinib 11-15	Ruxolitinib 16-20	Ruxolitinib 20+
Imatinib	Imatinib 1-5	Imatinib 6-10	Imatinib 11-15	Imatinib 16-20	Imatinib 20+
Dasatinib	Dasatinib 1-5	Dasatinib 6-10	Dasatinib 11-15	Dasatinib 16-20	Dasatinib 20+
Nilotinib	Nilotinib 1-5	Nilotinib 6-10	Nilotinib 11-15	Nilotinib 16-20	Nilotinib 20+
Bone marrow transplant	Bone marrow transplant 1-5	Bone marrow transplant 6-10	Bone marrow transplant 11-15	Bone marrow transplant 16-20	Bone marrow transplant 20+

Other (please specify)

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* 12. How many patients are still alive at the time of completing this survey?

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* 13. Many thanks for completing this survey. If you are able to help us with further collection of data, please supply your contact details below.

Name

Email Address