Diagnostic Algorithm for Patients with Eosinophil Disorders - Flowchart
Diagnostic Algorithm for Patients with Eosinophil Disorders - Flowchart Eosinophil CountHypereosinophilia and Hypereosinophilic Syndrome Eosinophil Count Hypereosinophilia and Hypereosinophilic Syndrome
«Flowchart»

Hypereosinophilia = HE

Hypereosinophilia = HE

Hypereosinophilia = HE

Hypereosinophilia = HE

End

End

End

End

+ Organ damage

+ Organ damage

+ Organ damage

+ Organ damage

Familial HES = HESF

Familial HES = HESF

Familial HES = HESF

Familial HES = HESF

F

Reactive HE = HER

Reactive HE = HER

Reactive HE = HER

Reactive HE = HER

R

Positive

Positive

Positive

Positive

Negative

Negative

Negative

Negative

+ Organ damage

+ Organ damage

+ Organ damage

+ Organ damage

Reactive HES = HESR

Reactive HES = HESR

Reactive HES = HESR

Reactive HES = HESR

R

Explore underlying cause
Establish the final diagnosis

Explore underlying cause
Establish the final diagnosis

Explore underlying cause
Establish the final diagnosis

Explore underlying cause
Establish the final diagnosis


WHO-based myeloid or lymphoid neoplasm with HE and abnormalities of PDGFRA, PDGFRB or FGFR1

WHO-based myeloid or lymphoid neoplasm with HE and abnormalities of PDGFRA, PDGFRB or FGFR1

WHO-based myeloid or lymphoid neoplasm with HE and abnormalities of PDGFRA, PDGFRB or FGFR1

WHO-based myeloid or lymphoid neoplasm with HE and abnormalities of PDGFRA, PDGFRB or FGFR1

PDGFRA, PDGFRB FGFR1

Screen for FIP1L1-PDGFRA by FISH or RT-PCR, and perform cytogenetic analysis for translocations involving 4q12 (PDGFRA), 5q31-q33 (PDGFRB), or 8p11-12 (FGFR1)

Screen for FIP1L1-PDGFRA by FISH or RT-PCR, and perform cytogenetic analysis for translocations involving 4q12 (PDGFRA), 5q31-q33 (PDGFRB), or 8p11-12 (FGFR1)

Screen for FIP1L1-PDGFRA by FISH or RT-PCR, and perform cytogenetic analysis for translocations involving 4q12 (PDGFRA), 5q31-q33 (PDGFRB), or 8p11-12 (FGFR1)

Screen for FIP1L1-PDGFRA by FISH or RT-PCR, and perform cytogenetic analysis for translocations involving 4q12 (PDGFRA), 5q31-q33 (PDGFRB), or 8p11-12 (FGFR1)

FIP1L1-PDGFRA PDGFRA PDGFRB FGFR1

Positive

Positive

Positive

Positive

Negative

Negative

Negative

Negative

Define the histopathological and clinical nature of the underlying neoplasm
Establish the final diagnosis

Define the histopathological and clinical nature of the underlying neoplasm
Establish the final diagnosis

Define the histopathological and clinical nature of the underlying neoplasm
Establish the final diagnosis

Define the histopathological and clinical nature of the underlying neoplasm
Establish the final diagnosis


+ Organ damage

+ Organ damage

+ Organ damage

+ Organ damage

Neoplastic HES = HESN

Neoplastic HES = HESN

Neoplastic HES = HESN

Neoplastic HES = HESN

N

Screen for other molecular markers and hematologic diseases

Screen for other molecular markers and hematologic diseases

Screen for other molecular markers and hematologic diseases

Screen for other molecular markers and hematologic diseases

WHO-defined myeloid neoplasm associated with eosinophilia

WHO-defined myeloid neoplasm associated with eosinophilia

WHO-defined myeloid neoplasm associated with eosinophilia

WHO-defined myeloid neoplasm associated with eosinophilia

Other molecular abnormality, clonal eosinophils, and/or increased marrow blasts (≥5-19%) ?

Other molecular abnormality, clonal eosinophils, and/or increased marrow blasts (≥5-19%) ?

Other molecular abnormality, clonal eosinophils, and/or increased marrow blasts (≥5-19%) ?

≥ Other molecular abnormality

Abnormal T-cell phenotype ± Th2 cytokine production ?

Abnormal T-cell phenotype ± Th2 cytokine production ?

Abnormal T-cell phenotype ± Th2 cytokine production ?

Abnormal T-cell phenotype ± Th2 cytokine production ?

Chronic eosinophilic leukemia, NOS

Chronic eosinophilic leukemia, NOS

Chronic eosinophilic leukemia, NOS

Chronic eosinophilic leukemia, NOS

HE of unknown significance = HEUS

HE of unknown significance = HEUS

HE of unknown significance = HEUS

HE of unknown significance = HEUS

US

Yes

Yes

Yes

Yes

No

No

No

No

+ Organ damage

+ Organ damage

+ Organ damage

+ Organ damage

Idiopathic HES = HESI

Idiopathic HES = HESI

Idiopathic HES = HESI

Idiopathic HES = HESI

I

Lymphocyte-variant hypereosinophilia

Lymphocyte-variant hypereosinophilia

Lymphocyte-variant hypereosinophilia

Lymphocyte-variant hypereosinophilia

Yes

Yes

Yes

Yes

No

No

No

No

+ Organ damage

+ Organ damage

+ Organ damage

+ Organ damage

HESL

HESL

HESL

HESL

L

Screen for secondary causes

Screen for secondary causes

Screen for secondary causes

Screen for secondary causes

Rare syndromes


Hereditary
Nonhereditary
Mono-organ

Rare syndromes


Hereditary
Nonhereditary
Mono-organ

Rare syndromes


Hereditary
Nonhereditary
Mono-organ


Hereditary
Nonhereditary
Mono-organ Rare syndromes

Familial HE = HEF

Familial HE = HEF

Familial HE = HEF

F Familial HE = HE(F)