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



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



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



WBC >25

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



?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



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



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