J Hematol

Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc

Journal website https://www.thejh.org

Short Communication

Volume 9, Number 3, September 2020, pages 71-78

Clinical Features of Plasmablastic Lymphoma: Case Series From an Asian Tertiary Cancer Center and Literature Review

Figure 1. Clinical course of patients with plasmablastic lymphoma. (a) Kaplan-Meier estimates for our cohort of 10 patients. The green line indicates OS, while the blue line indicates PFS. Five-year absolute survival estimates were given at 60% and 36% for OS and PFS respectively. (b) Swimmer plot illustrating a summary of the clinical courses taken by each of the 10 patients in our cohort. OS: overall survival; PFS: progression-free survival.

Figure 2. Repeated CRs to chemotherapy in a patient with relapsed stage IV plasmablastic lymphoma (case 7). (a) Contrast-enhanced CT illustrating CR of multiple solid cystic masses and peritoneal nodules following B-EPOCH as first-line therapy. (b) Contrast-enhanced T1-weighted MRI showing abnormal leptomeningeal enhancement at the cerebellar vermis and on the surface of the midbrain at level of the interpeduncular cistern, correlating with the presence of malignant blast cells on cerebrospinal fluid examination. Resolution of disease was observed following MPV as second-line therapy. (c) CR of enhancing soft-tissue masses in the pelvic cavity following third-line chemotherapy with the ICE regimen. Red arrows indicate initial sites of disease. CRs: complete responses; CT: computed tomography; B-EPOCH: bortezomib plus etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin; MRI: magnetic resonance imaging; MPV: methotrexate, procarbazine, vincristine; ICE: ifosfamide, carboplatin, etoposide.

Figure 3. FDG-PET/CT imaging features in plasmablastic lymphoma. (case 1) FDG-avid soft tissue mass (SUVmax 21.5) in the oral cavity in partial response following B-CVP and consolidation radiotherapy. (case 9) Locally-advanced FDG-avid anorectal mass (SUVmax 30.9) in complete metabolic remission after EPOCH and consolidation radiotherapy. (case 10) Complete metabolic response of initially FDG-acid posterior chest wall mass (SUVmax 28.2) after EPOCH and radiotherapy. Red arrows indicate initial sites of disease. FDG-PET/CT: 18-fluorodeoxyglucose positron emission tomography/computed tomography; B-CVP: bortezomib, cyclophosphamide, vincristine, prednisone; EPOCH: etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin.

**Table 1.** Clinical Characteristics and Treatment Outcomes of Patients With Plasmablastic Lymphoma
Parameter	Case 1	Case 2	Case 3	Case 4	Case 5	Case 6	Case 7	Case 8	Case 9	Case 10
^aAssessed using Hodgkin Disease Ann Arbor Staining Classification criteria; ^bAssessed using the International Non-Hodgkin Lymphoma Prognostic Factors Project’s IPI score. ECOG: Eastern Cooperative Oncology Group performance status; IPI: International Prognostic Index; NA: not available; B-CVP: bortezomib, cyclophosphamide, vincristine, prednisone; EPOCH: etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin; CHOP: cyclophosphamide, doxorubicin, vincristine, prednisolone; B-EPOCH: bortezomib plus EPOCH.
Age (years)	61	69	59	43	54	43	33	91	50	55
Sex	Male	Male	Male	Male	Male	Male	Female	Male	Male	Male
Ethnicity	Malay	Chinese	Chinese	Chinese	Chinese	Chinese	Chinese	Chinese	Chinese	Malay
ECOG status	1	1	0	1	0	0	2	1	0	0
Immune deficiency	Renal transplant	NIL	HIV/AIDS (pre-existing)	HIV/AIDS (at diagnosis)	Renal transplant	HIV/AIDS (at diagnosis)	HIV/AIDS (pre-existing)	NIL	HIV/AIDS (at diagnosis)	NIL
CD4 counts (cells per mm³)	NA	NA	139	153	NA	7	68	NA	17	NA
Smoking history	No	Yes	No	No	No	No	Yes	No	No	Yes
B-symptoms	No	Yes	No	Yes	Yes	Yes	No	No	No	Yes
Stage^a	IE	I	IIE	IV	IIE	IV	IV	IE	IIIE	IE
Nodal involvement	No	Mediastinal	Mesenteric	Cervical; parotid; retropharyngeal; tonsils; axilla; mediastinal; hilar; retroperitoneal; iliac; inguinal	Cervical; supraclavicular; axilla; mediastinal	Supraclavicular; axilla; mediastinal; hilar; retroperitoneal; iliac	Mediastinal; axilla	No	Supraclavicular; axilla; splenic hilum; periportal; retroperitoneal; iliac	No
Extranodal site	Oral cavity	No	Bowel	Liver; posterior; nasal space	Base of tongue	Liver; lung; bone marrow	Adnexa; peritoneal	Posterior nasal space	Rectum	Chest wall
IPI^b	1	2	1	3	1	3	4	1	2	1
Initial therapy	B-CVP	EPOCH	EPOCH	EPOCH	CHOP	Supportive care only	B-EPOCH	Radiation only	EPOCH; radiation	EPOCH; radiation
Treatment response	Partial response	Complete response	Complete response	Partial response	Partial response	NA	Complete response	Complete response	Complete response	Complete response
Cause of death	Pneumonia	NA	NA	Disease	Disease	Pneumonia	NA	NA	NA	NA
Overall survival (months)	6.7	74.6	184.3	6.4	6.8	0.8	102.3	37.5	26.8	12.0

Table 1. Clinical Characteristics and Treatment Outcomes of Patients With Plasmablastic Lymphoma

Parameter

Case 1

Case 2

Case 3

Case 4

Case 5

Case 6

Case 7

Case 8

Case 9

Case 10

^aAssessed using Hodgkin Disease Ann Arbor Staining Classification criteria; ^bAssessed using the International Non-Hodgkin Lymphoma Prognostic Factors Project’s IPI score. ECOG: Eastern Cooperative Oncology Group performance status; IPI: International Prognostic Index; NA: not available; B-CVP: bortezomib, cyclophosphamide, vincristine, prednisone; EPOCH: etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin; CHOP: cyclophosphamide, doxorubicin, vincristine, prednisolone; B-EPOCH: bortezomib plus EPOCH.

Age (years)

Sex

Male

Female

Male

Ethnicity

Malay

Chinese

Malay

ECOG status

Immune deficiency

Renal transplant

NIL

HIV/AIDS (pre-existing)

HIV/AIDS (at diagnosis)

Renal transplant

HIV/AIDS (at diagnosis)

HIV/AIDS (pre-existing)

NIL

HIV/AIDS (at diagnosis)

NIL

CD4 counts
(cells per mm³)

139

153

Smoking history

Yes

B-symptoms

Yes

Stage^a

IIE

IIIE

Nodal involvement

Mediastinal

Mesenteric

Cervical; parotid; retropharyngeal; tonsils; axilla; mediastinal; hilar; retroperitoneal; iliac; inguinal

Cervical; supraclavicular; axilla; mediastinal

Supraclavicular; axilla; mediastinal; hilar; retroperitoneal; iliac

Mediastinal; axilla

Supraclavicular; axilla; splenic hilum; periportal; retroperitoneal; iliac

Extranodal site

Oral cavity

Bowel

Liver; posterior; nasal space

Base of tongue

Liver; lung; bone marrow

Adnexa; peritoneal

Posterior nasal space

Rectum

Chest wall

IPI^b

Initial therapy

B-CVP

EPOCH

CHOP

Supportive care only

B-EPOCH

Radiation only

EPOCH; radiation

Treatment response

Partial response

Complete response

Partial response

Complete response

Cause of death

Pneumonia

Disease

Pneumonia

Overall survival (months)

6.7

74.6

184.3

6.4

6.8

0.8

102.3

37.5

26.8

12.0

**Table 2.** Hematological, Biochemical and Histopathological Profiles
Parameter	Case 1	Case 2	Case 3	Case 4	Case 5	Case 6	Case 7	Case 8	Case 9	Case 10
^aPositive staining for MUM-1/OCT2/BOB1/CD45 in keeping with plasmablastic differentiation. LDH: lactate dehydrogenase; EBER: Epstein-Barr virus-encoded RNA; ANC: absolute neutrophil count; ALC: absolute lymphocyte count.
Hemoglobin (12 - 16 g/dL)	9.3	13.0	12.6	10.5	11.2	6.4	12.3	14.0	12.6	7.1
Leucocyte (4 - 10 × 10⁹/L)	5.6	13.3	14.3	13.6	6.9	7.6	9.5	4.4	5.1	33.0
Platelet (140 - 440 × 10⁹/L)	85	188	543	289	320	163	36	218	256	1067
ANC (2.0 - 7.5 × 10⁹/L)	4.4	10.1	12.4	10.1	30.5	6.6	6.9	2.5	4.0	28.3
ALC (1.0 - 3.0 × 10⁹/L)	0.6	1.7	1.3	0.1	1.2	0.6	1.1	1.1	0.4	3.1
Albumin (40 - 51 g/L)	28	26	27	20	33	18	12	39	37	20
Raised LDH	No	Yes	Yes	Yes	Yes	Yes	Yes	No	Yes	Yes
EBER	Present	Present	Present	Present	Present	Present	Present	Present	Present	Present
CD20	Absent	Absent	Absent	Absent	Absent	Absent	Absent	Absent	Absent	Absent
CD79a	Absent	Present	Absent	Absent	Not tested	Not tested	Present	Present	Present	Absent
CD30	Present	Absent	Present	Present	Not tested	Present	Not tested	Absent	Absent	Present
CD38	Absent	Not tested	Absent	Present	Not tested	Present	Not tested	Not tested	Present	Present
CD138	Absent^a	Present	Present	Absent	Present	Absent	Present	Present	Present	Absent
MUM-1	Present	Present	Present	Present	Present	Present	Present	Not tested	Present	Present
Ki-67 (%)	80 - 90	70	98	> 95	70 - 80	40 - 50	> 80	60 - 70	> 90	90

Table 2. Hematological, Biochemical and Histopathological Profiles

Parameter

Case 1

Case 2

Case 3

Case 4

Case 5

Case 6

Case 7

Case 8

Case 9

Case 10

^aPositive staining for MUM-1/OCT2/BOB1/CD45 in keeping with plasmablastic differentiation. LDH: lactate dehydrogenase; EBER: Epstein-Barr virus-encoded RNA; ANC: absolute neutrophil count; ALC: absolute lymphocyte count.

Hemoglobin (12 - 16 g/dL)

9.3

13.0

12.6

10.5

11.2

6.4

12.3

14.0

12.6

7.1

Leucocyte (4 - 10 × 10⁹/L)

5.6

13.3

14.3

13.6

6.9

7.6

9.5

4.4

5.1

33.0

Platelet (140 - 440 × 10⁹/L)

188

543

289

320

163

218

256

1067

ANC (2.0 - 7.5 × 10⁹/L)

4.4

10.1

12.4

10.1

30.5

6.6

6.9

2.5

4.0

28.3

ALC (1.0 - 3.0 × 10⁹/L)

0.6

1.7

1.3

0.1

1.2

0.6

1.1

0.4

3.1

Albumin (40 - 51 g/L)

Raised LDH

Yes

EBER

Present

CD20

Absent

CD79a

Absent

Present

Absent

Not tested

Present

Absent

CD30

Present

Absent

Present

Not tested

Present

Not tested

Absent

Present

CD38

Absent

Not tested

Absent

Present

Not tested

Present

Not tested

Present

CD138

Absent^a

Present

Absent

Present

Absent

Present

Absent

MUM-1

Present

Not tested

Present

Ki-67 (%)

80 - 90

> 95

70 - 80

40 - 50

> 80

60 - 70

> 90

**Table 3.** Glossary of Asian Studies of Plasmablastic Lymphoma
Study	Year	Enrolment country	Type of study	Sample size (n)	% with HIV	% with EBV	Median OS (months)
EBV: Epstein-Barr virus; OS: overall survival.
Miao et al [12]	2020	Asia	Literature review	98	27% (10/37)	56.8% (21/37)	11.4
	China	Case series	13	7.7% (1/13)	61.5% (8/13)	11.3
Han et al [11]	2017	China	Literature review	60	0% (0/60)	46.8% (22/47)	7
Wang et al [13]	2016	China	Case series	6	100% (6/6)	66.7% (4/6)	-
Rudresha et al [14]	2017	India	Case series	13	61.5% (8/13)	-	9 (HIV⁺), 6 (HIV^-)
Chen et al [15]	2018	Taiwan	Case series	26	14.3% (1/7)	40% (4/10)	3
Liu et al [16]	2012	Japan	Case series	10	0%	100% (10/10)	-
Suzuki et al [17]	2010	Japan	Literature review	10	10% (1/10)	50% (1/2, only two tested)	-
Kim et al [18]	2009	Korea	Case series	6	0% (0/6)	16.7% (1/6)	5.3

Table 3. Glossary of Asian Studies of Plasmablastic Lymphoma

Study

Year

Enrolment country

Type of study

Sample size (n)

% with HIV

% with EBV

Median OS (months)

EBV: Epstein-Barr virus; OS: overall survival.

Miao et al [12]

2020

Asia

Literature review

27% (10/37)

56.8% (21/37)

11.4

China

Case series

7.7% (1/13)

61.5% (8/13)

11.3

Han et al [11]

2017

China

Literature review

0% (0/60)

46.8% (22/47)

Wang et al [13]

2016

China

Case series

100% (6/6)

66.7% (4/6)

Rudresha et al [14]

2017

India

Case series

61.5% (8/13)

9 (HIV⁺), 6 (HIV^-)

Chen et al [15]

2018

Taiwan

Case series

14.3% (1/7)

40% (4/10)

Liu et al [16]

2012

Japan

Case series

100% (10/10)

Suzuki et al [17]

2010

Japan

Literature review

10% (1/10)

50% (1/2, only two tested)

Kim et al [18]

2009

Korea

Case series

0% (0/6)

16.7% (1/6)

5.3