PRIMARY PLASMA CELL LEUKEMIA: YET A DIAGNOSTIC CHALLENGE, REPORT OF TWO
POSTER 38
CLINICAL CASES.
Liri Seraj1 and Arjan Pushi2
(1)Hematology Specialty, Department of Internal Medicine, University Hospital Center Mother Teresa
Tirana, University of Medicine of Tirana, Tirana, Albania
(2)Hematology Department, University Hospital Center Mother Teresa Tirana, University of Medicine
of Tirana, Tirana, Albania
Background
PCL is defined as primary when a “de-novo” diagnosis on a patient, not previously diagnosed as
multiple myeloma (MM), is made. Plasma Cell Leukemia (PCL) is defined as the presence of ≥20% of
circulating plasma cells (CPCs) on differential count of the leukocytes and an absolute plasma cell
count of ≥2x109/L in the peripheral blood. It is a rare and severely aggressive malignancy.
Case 1 summary
A 63 years old female patient with one-month history of malaise, abdominal cramps, skeletal pain,
weight loss and weakness. Upon admission the hematologic values were: white blood cells
26.900/mm3, red blood cells 3.720.000/mm3, hemoglobin 10.8g/dL, counted platelets (cPLT)
171.000/mm3, smear description showed many groups of plasma cells. Leukocytes differential
showed 39% plasma cells. Bone Marrow Aspirate showed plasma cells 41% with the characteristics of
plasmablasts. The flow cytometry results showed 35% atypical cells expressing CD38 (100% positive),
CD13 (30% positive), CD33 (40% positive), cKAPPA (90% positive). On CT scan no bone lesions were
evident. A diagnosis of pPCL was made and vincristine, cyclophosphamine, dexamethasone was
started. The patient died three months later.
Case 2 summary
A 64 years old male patient presented with complains of bone pain, gradually worsening shortness of
breath over the past week, malaise and fatigue over the past month. Upon admission the
hematologic values were: white blood cells 4.400/mm3, red blood cells 2.540.000/mm3, hemoglobin
7.4 g/dl. Differential of the white blood cells showed 20% circulating plasma cells with a nucleus with
fine stippled chromatin. A bone marrow aspiration revealed 25% plasma cells with fine stippled
chromatin resembling more to plasmablasts. Flow cytometry showed 20% of atypical cells which
expressed CD38 (90% positive), CD138 (90% positive). No bone lesions were evident on CT scan. A
diagnosis of pPCL was made and bortezomib, cyclophosphamide, dexamethasone was started. The
patient died two months later.
Conclusions
Considering both clinical cases, especially the 2nd one, we suggest that the threshold of 20% of CPCs
and an absolute count of ≥2x109/L of CPCs should be revised in order to develop accurate diagnostic
criteria. We suggest that one of the revised criteria, instead of both of them, be met in order to
determine the diagnosis of pPCL.
Abbreviations: pPCL- primary plasma cell leukemia, CD-clusters of differentiation.
SCIENTIFIC
PROGRAMME
RARE SUBSETS OF
ACUTE LEUKAEMIA
TRACKING LEUKAEMIC
STEM CELLS (LSCs)
ROUTINE DIAGNOSIS
GENE EXPRESSION
AND MUTATIONAL
PROFILING
DEBATE 1 – ALL
PATIENTS WITH
INTERMEDIATE-RISK
AML MUST BE
TRANSPLANTED
INTERACTIVE
CASES 1 – MUTATION-BASED
THERAPY
OFF-LABEL
ROUNDTABLE –
SHOULD WE REALLY
USE NEW TARGETED
INHIBITORS AS SINGLE
AGENTS ?
ADDITION OF A 3RD
AGENT TO FRONTLINE
7+3
ROUNDTABLE –
CURATIVE OPTIONS
FOR OLDER AML
INTERACTIVE CASES 2
DEBATE 2 - BEST
TREATMENT FOR
NPM1-MUTATED AML IN
THE NEXT FUTURE ?
ALLOGENEIC
HAEMATOPOIETIC
STEM CELL
TRANSPLANTATION
(HSCT)
IMMUNOTHERAPY FOR
ACUTE LEUKAEMIA
DEBATE 3 - T-ALL:
WHERE ARE WE GOING
NOW?
SELECTED ABSTRACTS
AND CLINICAL
CASES FOR AN ORAL
PRESENTATION
SELECTED ABSTRACTS
FOR A POSTER
PRESENTATION
DISCLOSURES