POSTER 1
ACUTE LYMPHOBLASTIC LEUKEMIC TRANSFORMATION FROM PRIMARY
MYELOFIBROSIS
Carolina Afonso1, Rita Gomes1, Sara Duarte1, Bárbara Marques2, José Pedro Carda2 and Letícia Ribeiro3
(1)Clinical Hematology Department , Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
(2)Faculty of Medicine of University of Coimbra, Coimbra, Portugal
(3)Coimbra Institute for Clinical and Biomedical Research ( iCBR ), Faculty of Medicine of University of
Coimbra, Coimbra, Portugal
Introduction
The reported frequency of blast transformation in primary myelofibrosis (PMF) is 5―30%.
Leukemic transformation (LT) usually occurs in the form of acute myeloid leukemia, being
lymphoblastic transformation a very rare event. We report a case of acute lymphoblastic LT in
a patient with PMF.
Case Report
An asymptomatic 66-year-old man was evaluated in November 2013 for mild microcytosis
(mean corpuscular volume 77.5 fL, reference range 80-100 fL) and elevated lactic acid
dehydrogenase level (632 U/L, reference range < 248 U/L) over the previous 12 months; blood
counts were normal and peripheral blood (PB) smear showed mild anisocytosis with
microcytosis; ferritin level was normal (223 ng/mL, reference range 21-275 ng/mL), and
soluble transferrin receptor/log ferritin index was < 1, being highly unsuggestive of iron
deficiency. Physical examination was normal.
Bone marrow biopsy was performed and revealed granulocytic, erythroid and megakaryocytic
hyperplasia, as well as grade 1 reticulin fibrosis, consistent with cellular/prefibrotic phase of
PMF. JAK2 V617F, CALR exon 9 and MPL exon 10 mutations were not detected.
A diagnosis of triple-negative PMF was established (intermediate-1 risk disease according to
the Dynamic International Prognostic Scoring System, DIPSS). The patient was initially
observed without treatment. However, approximately 24 months later, treatment with
ruxolitinib was initiated due to progressive spleen growth. After 18 months on ruxolitinib
therapy, the patient exhibited signs of clinical deterioration, with worsening splenomegaly
and constitutional symptoms, as well as progressive anemia and thrombocytopenia.
Simultaneously, he developed leukocytosis and a rapid increase of PB blast count (up to 25%
in the PB), predicting LT. Flow cytometric immunophenotyping was performed on PB and
revealed the presence of two abnormal clones, one of myeloid and the other one of lymphoid
origin. The latter expressed CD10 (bright), CD19, CD20 and CD22, consistent with common B
cell acute lymphoblastic leucemia. Karyotype was normal and BCR-ABL fusion transcript was
undetected.
At this time, the patient was 72 years old and had a poor performance status (ECOG
performance status of 3), thus being unfit for intensive chemotherapy. Treatment with
hydroxyurea and low-dose prednisolone was initiated with palliative intent, and ruxolitinib
slowly tapered off. The patient's condition rapidly deteriorated, and he died 6 months later.
Conclusion
LT from PMF is associated with an agressive course and poor overall survival. Myeloblastic
SCIENTIFIC PROGRAMME
SESSION I
OPTIMIZING
CYTOREDUCTION
SESSION II
MANAGEMENT OF CML
WITH TKI
SESSION III
MPN RISK
STRATIFICATION
INCLUDING VASCULAR
EVENTS
DEBATE 1
INTERFERON ALPHA
SHOULD BE FRONT LINE
THERAPY IN ALL ET/PV
PATIENTS
ROUNDTABLE 1
INFECTIONS IN
MYELOPROLIFERATIVE
DISORDERS, INCLUDING
CML
ROUNDTABLE 2
PREGNANCY AND
PARENTING
DEBATE 2
ALLOGENEIC STEM CELL
TRANSPLANTATION
SHOULD BE CONSIDERED
THIRD LINE OPTION IN
CHRONIC PHASE CML
SESSION IV
EVOLVING THERAPIES
IN MYELOFIBROSIS
SESSION V
MANAGEMENT OF
ADVANCED AND UNUSUAL
DISEASE (MPN AND CML)
SESSION VI
TREATMENT FREE
REMISSION IN CML
KEYNOTE LECTURE
SELECTED ABSTRACTS
FOR AN ORAL
PRESENTATION
SELECTED ABSTRACTS
FO R A POSTER
PRESENTATION
DISCLOSURES