Rare Leukaemias

 

 

B cell Prolymphocytic Leukaemia (B-PLL)

*** B-PLL has been removed as an entity in the WHO-HAEM5 2022 classification. These patients will fit best either in ‘Prolymphocytic Progression of CLL’ or ‘Splenic B-Cell Lymphoma/Leukaemia with prominent nucleoli’, depending on the individual case. I have not deleted this section yet as will take some time for these changes to filter through. ***

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 with >80% of cells being mature neutrophils/band forms

CSF3R mutation present in >60% of cases

 

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