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| |Hairy cell leukaemia | | |Hairy cell leukaemia |
| |} | | |} |
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| ==Definition / Description of Disease==
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|
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| *Hairy cell leukemia (HCL) is a rare indolent neoplasm of B-cell origin seen mostly in adults
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| *Name derives from the the hair-like projections of the cytoplasm that surround the cells
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| *Hairy cells most closely resemble mature lymphoid cells
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| *Marked splenomegaly and marrow infiltration result in left flank discomfort, fatigue, and susceptibility to infections
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| *Most respond well to monotherapy with a purine analog or interferon alpha
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|
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| ==Synonyms / Terminology==
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|
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| *Leukemic reticuloendotheliosis
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|
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| ==Epidemiology / Prevalence==
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|
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|
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| *Incidence (age adjusted) ~ 0.3/100,000
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| *2% of lymphoid leukemias
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| *Median age: 58 years, rarely in patients in their 20s
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| *Males:Females: 4:1
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| *Whites >> Blacks
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| *78% to 92% 5yr survival
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| *
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| <blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref>{{Cite journal|last=Lr|first=Teras|last2=Ce|first2=DeSantis|last3=Jr|first3=Cerhan|last4=Lm|first4=Morton|last5=A|first5=Jemal|last6=Cr|first6=Flowers|date=2016|title=2016 US Lymphoid Malignancy Statistics by World Health Organization Subtypes|url=https://pubmed.ncbi.nlm.nih.gov/27618563/|language=en|pmid=27618563}}</ref><blockquote class="blockedit">
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| <center><span style="color:Maroon">'''End of V4 Section'''</span>
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| ----
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| </blockquote>
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| ==Clinical Features==
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| {| class="wikitable"
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| |'''Signs and Symptoms'''
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| *Asymptomatic (incidental finding on complete blood counts)
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| *B-symptoms (weight loss, fever, night sweats)
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| *Fatigue
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| *Splenic enlargement and discomfort
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| *Recurrent infections
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| *Lymphadenopathy (rare)
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| |-
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| |'''Laboratory Findings'''
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| |Pancytopenia (monocytopenia is characteristic)
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| Lymphocytosis (low level)
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| |}
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|
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| <blockquote class="blockedit">{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}</blockquote>
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|
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|
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| '''Signs & Symptoms'''
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|
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| *Asymptomatic (incidental finding on complete blood counts)
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| *B-symptoms (weight loss, fever, night sweats)
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| *Fatigue
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| *LUQ pain (splenomegaly)
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| *Lymphadenopathy (uncommon)
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|
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| '''Laboratory findings'''
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|
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| *Cytopenias
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| *Monocytopenia
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| *Lymphocytosis (low level)
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| <blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref name=":2">{{Cite journal|last=Grever|first=Michael R.|last2=Abdel-Wahab|first2=Omar|last3=Andritsos|first3=Leslie A.|last4=Banerji|first4=Versha|last5=Barrientos|first5=Jacqueline|last6=Blachly|first6=James S.|last7=Call|first7=Timothy G.|last8=Catovsky|first8=Daniel|last9=Dearden|first9=Claire|date=2017|title=Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia|url=https://ashpublications.org/blood/article/129/5/553/36153/Consensus-guidelines-for-the-diagnosis-and|journal=Blood|language=en|volume=129|issue=5|pages=553–560|doi=10.1182/blood-2016-01-689422|issn=0006-4971|pmc=PMC5290982|pmid=27903528}}</ref><blockquote class="blockedit">
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| <center><span style="color:Maroon">'''End of V4 Section'''</span>
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| ----
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| <blockquote class="blockedit">
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| <center><span style="color:Maroon">'''End of V4 Section'''</span>
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| ----
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| </blockquote>
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| </blockquote>
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| ==Sites of Involvement==
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|
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| *Spleen (red pulp)
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| *Bone marrow
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| *Liver
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| *Blood (small number)
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| <blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref name=":2" /><blockquote class="blockedit">
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| <center><span style="color:Maroon">'''End of V4 Section'''</span>
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| ----
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| </blockquote>
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| ==Morphologic Features==
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|
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|
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| *Small lymphoid cells
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| *Abundant pale blue-grey lacey cytoplasm
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| *Ovoid nuclei ± indentation or folding
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| *Inconspicuous nucleoli
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| *Circumferential hairy projections (smear preparations)
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| *"Fried egg" appearance of cells (tissue sections)
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| *Marrow (reticulin) fibrosis
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|
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|
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| <blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref name=":2" /><blockquote class="blockedit">
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| <center><span style="color:Maroon">'''End of V4 Section'''</span>
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| ----
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| </blockquote>
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| ==Immunophenotype==
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|
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| {| class="wikitable sortable"
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| |-
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| !Finding!!Marker
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| |-
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| |Positive (B-cell lineage markersl)||CD19, CD20 (bright), CD22, PAX5, FMC7, sIg (bright, monoclonal)
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| |-
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| |Positive||CD200 (bright), CD11c, CD103, CD123, CD25, CD72, annexin‐A1, BRAF V600E, phospho-ERK
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| |-
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| |Negative||CD5, CD10 (10-20% may be positive), CD23, CD27
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| |}
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| <blockquote class="blockedit">{{Box-round|title=v4:Immunophenotype|The content below was from the old template. Please incorporate above.}}</blockquote>
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| {| class="wikitable"
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| |-
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| !Finding!!Marker
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| |-
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| |Positive (B-cell lineage markers)||CD19, CD20 (bright), CD22, PAX5, FMC7, sIg (bright, monoclonal)
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| |-
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| |Positive
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| |CD200 (bright), CD11c, CD103, CD123, CD25, CD72, annexin‐A1, BRAF V600E, phospho-ERK
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| |-
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| |Negative||CD5, CD10 (10-20% may be positive), CD23, CD27
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| |}
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|
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|
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| <blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref name=":2" /><blockquote class="blockedit">
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| <center><span style="color:Maroon">'''End of V4 Section'''</span>
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| ----
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| <blockquote class="blockedit">
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| <center><span style="color:Maroon">'''End of V4 Section'''</span>
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| ----
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| </blockquote>
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| </blockquote>
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| ==WHO Essential and Desirable Genetic Diagnostic Criteria== | | ==WHO Essential and Desirable Genetic Diagnostic Criteria== |
| <span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span> | | <span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span> |
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| ==Additional Information== | | ==Additional Information== |
| | This disease is <u>defined/characterized</u> as detailed below: |
| | |
| | *Hairy cell leukemia (HCL) is a rare indolent neoplasm of B-cell origin seen mostly in adults |
| | *Name derives from the the hair-like projections of the cytoplasm that surround the cells |
| | *Hairy cells most closely resemble mature lymphoid cells |
| | *Marked splenomegaly and marrow infiltration result in left flank discomfort, fatigue, and susceptibility to infections |
| | *Most respond well to monotherapy with a purine analog or interferon alpha |
| | |
| | The <u>epidemiology/prevalence</u><ref name=":1" /><ref>{{Cite journal|last=Lr|first=Teras|last2=Ce|first2=DeSantis|last3=Jr|first3=Cerhan|last4=Lm|first4=Morton|last5=A|first5=Jemal|last6=Cr|first6=Flowers|date=2016|title=2016 US Lymphoid Malignancy Statistics by World Health Organization Subtypes|url=https://pubmed.ncbi.nlm.nih.gov/27618563/|language=en|pmid=27618563}}</ref> of this disease is detailed below: |
| | |
| | *Incidence (age adjusted) ~ 0.3/100,000 |
| | *2% of lymphoid leukemias |
| | *Median age: 58 years, rarely in patients in their 20s |
| | *Males:Females: 4:1 |
| | *Whites >> Blacks |
| | *78% to 92% 5yr survival |
| | |
| | The <u>clinical features</u><ref name=":1" /><ref name=":2">{{Cite journal|last=Grever|first=Michael R.|last2=Abdel-Wahab|first2=Omar|last3=Andritsos|first3=Leslie A.|last4=Banerji|first4=Versha|last5=Barrientos|first5=Jacqueline|last6=Blachly|first6=James S.|last7=Call|first7=Timothy G.|last8=Catovsky|first8=Daniel|last9=Dearden|first9=Claire|date=2017|title=Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia|url=https://ashpublications.org/blood/article/129/5/553/36153/Consensus-guidelines-for-the-diagnosis-and|journal=Blood|language=en|volume=129|issue=5|pages=553–560|doi=10.1182/blood-2016-01-689422|issn=0006-4971|pmc=PMC5290982|pmid=27903528}}</ref> of this disease are detailed below: |
| | |
| | * Signs and symptoms - Asymptomatic (incidental finding on complete blood counts); B-symptoms (weight loss, fever, night sweats); Fatigue; Splenic enlargement and discomfort; Recurrent infections; Lymphadenopathy (rare) |
| | * Laboratory findings - Pancytopenia (monocytopenia is characteristic); Lymphocytosis (low level) |
| | |
| | The <u>sites of involvement</u><ref name=":1" /><ref name=":2" /> of this disease are detailed below: |
| | |
| | *Spleen (red pulp); Bone marrow; Liver; Blood (small number) |
| | |
| | The <u>morphologic features</u><ref name=":1" /><ref name=":2" /> of this disease are detailed below: |
| | |
| | *Small lymphoid cells; Abundant pale blue-grey lacey cytoplasm; Ovoid nuclei ± indentation or folding; Inconspicuous nucleoli; Circumferential hairy projections (smear preparations); "Fried egg" appearance of cells (tissue sections); Marrow (reticulin) fibrosis |
| | |
| | The <u>immunophenotype</u><ref name=":1" /><ref name=":2" /> of this disease is detailed below: |
| | |
| | Positive ((B-cell lineage markers) - |
| | |
| | Positive - CD19, CD20 (bright), CD22, PAX5, FMC7, sIg (bright, monoclonal) |
| | |
| | Positive - CD200 (bright), CD11c, CD103, CD123, CD25, CD72, annexin‐A1, BRAF V600E, phospho-ERK |
|
| |
|
| *N/A
| | Negative - CD5, CD10 (10-20% may be positive), CD23, CD27 |
|
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| ==Links== | | ==Links== |
