Rare Leukaemias

 

 

B cell Prolymphocytic Leukaemia (B-PLL)

 

1% of lymphocytic leukaemias

Median age 60-70, M=F

High WBC, B Symptoms, Massive Splenomegaly

50% of patients thrombocytopenic / anaemic

Aggressive disease, median survival 3 years from diagnosis

 

Diagnosis

Film – Prolymphocytes must be >55% of lymphoid cells seen, usually >90%

Immunophenotype – CD19+, CD20+, CD22+, SmIg++, CD5+/-, CD23-, SOX11-, CD1-

Cytogenetics – 17p del in 50%, C-MYC, TP53 mutation

 

Treatment

Difficult, poor responses

FCR / R-Benda / Ibrutinib / Alemtuzumab

 

Chronic Eosinophilic Leukaemia (CEL)

See Eosinophilia

 

Chronic Neutrophilic Leukaemia (CNL)

 

Rare myeloproliferative neoplasm

Persistent neutrophilic leucocytosis & hepatosplenomegaly

 

Diagnosis

WBC >25

CSF3R mutation present in most cases, ?diagnostic and to be included in next WHO

 

Treatment

?Ruxolitinib – Phase 2 study in CNL and aCML. 66% response rate for CNL.

 

Hairy Cell Leukaemia (HCL)

 

See Hairy Cell Leukaemia

Pure Erythroid Leukaemia

 

Extremely rare

Any age including childhood

Usually progression from MDS but can occur de novo

Median survival only 3 months

 

Diagnosis

BM aspirate - >80% of cells are erythroid, with >30% proerythroblasts and no significant myeloblast presence.

 

Treatment

As for AML

 

T cell Large Granular Lymphocytic Leukaemia (T-LGL)

 

See T & NK Neoplasms

 

T cell Prolymphocytic Leukaemia (T-PLL)

 

See T & NK Neoplasms