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 http://www.thejh.org

Case Report

Volume 3, Number 1, March 2014, pages 13-18

Erythroblastic Synartesis in a Patient With Low Grade B Cell Lymphoma

Figure 1. Severe microcytic, hypochromic anemia with marked poikilocytosis and numerous nucleated red blood cells with dysplastic changes including bi-nucleation.

Figure 3. Multiple examples of erythroblastic synartesis or syncytial-like cluster of erythroblasts linked together by close apposition of their plasma membranes forming a nonbasophilic cytoplasmic clear zone. There is extreme erythroid hyperplasia with marked dyserythropoiesis (unilineage dysplasia).

**Table 1.** The Clinically Salient Features of the Published Reports
Author	Year	Age	sex	Hgb	M -protein	Other Labs	Underlying disease	Therapy	Long term Outcome
Abbreviations: ACLA: anti cardiolipin antibody; AN A: antineutrophil antibodies; CLL: chronic lymphocytic leukemia; ESR: erythrocyte sedimentation rate; F: female; Hgb: hemoglobin; LDH: lactate dehydrogenase; M: Male; NR: not reported; OSH: outside hospital; Plt: platelets; ppx: prophylactic; T-bili: total bilirubin; TD: transfusion dependent; WBC: white blood cell count.
Gorius [1]	1973	74	F	7.75 (Transfusion dependent)	NR	Serum iron 105, TIBC 240, T bili 1.3, Coombs negative, Retic count 0.5%, Negative HAM’s test, no agglutination with anti-i.	NR	NR	NR
Nagao [3]	1976	29	F	NR	NR	Hematocrit 24%, Plt 263 × 10⁴/cm², WBC 5,900/cm², retic count < 1‰	NR	NR	NR
Cramer [4]	1999	24	F	6	NR	Reticulocyte count reduced; nl serum iron;	Small lymphocytic lymphoma	Chlorambucil → no response; corticosteroids → decreased transfusion requirements, anemia recurred with taper; multiagent chemotherapy → hgb stabilized	Died from Richter transformation after 10 years.
		70	F	5.5	IgG, kappa 3 g/L	Iron 35, ferritin 175, LDH 667, bilirubin 9.3, haptoglobin markedly reduced, Retic count 20 × 10⁹/L Coombs positive for complement, lupus anticoagulant present, ACLA positive	Sjogren’s syndrome	Corticosteroids with resolution of anemia, but relapse with withdrawal of steroids	NR
		46	F	7.9	IgG, kappa (1.7 g/L)	WBC 18.7 with 76% lymphs,	CLL	6 cycles low dose CHOP, ppx IVIG, 1 year chlorambucil → remission (hgb 12.7); relapsed 6 months later with hgb 6.9; fludarabine 6 mo later with transient incomplete remission	Died from septic cellulitis 3 years later
Bacher [6]	2004	74	M	9	IgG, kappa (3.3 g/dL)	ESR 75, ANA neg, AMA neg, C4 < 0.01, C3 0.52 (low), IgG 35.4 (elevated), No signs of hemolysis, Retic count 15‰.	None	None (In good clinical condition)	NR
Liapis [7]	2008	70	NR	4.9	IgG, kappa (1.5 g/L)	Reticulocyte count 1.5 × 10⁹/L; ferritin, B12, and folate nl.	Small lymphocytic lymphoma	Therapy (not specified) of small lymphoctic lymphoma lead to resolution	NR
Zaninoni [5]	2010	44	M	9	NR	Reduced reticulocytes (20 × 10⁹/L), unconjugated bili 1.8, LDH 550, haptoglobin < 20, DAT negative	NR	Steroids and cyclosporine “with some response” at OSH; Repeat prednisone with clinical response. Relapsed 2 years later and again treated with prednisone taper with good response.	NR
Papakonstantinou [8]	2010	35	M	8.1 (Transfusion dependent)	None	WBC 2.8 (29% PMN, 53% lymphocytes), retic count 105.2 × 10⁹ L; LDH 355, IgA undetectable, CD19+ B-lymphocytes reduced (21 × 10⁶/L); no monoclonal B-cell population detected. Serum IgG, IgM, haptoglobin, vitamin B12, folic acid, ferritin, and B2-microglobulin were normal. Coombs negative.	None	Prednisolone with no response, IVIG with no response; complete remission with rituximab	Response maintained at 30 months.

Table 1. The Clinically Salient Features of the Published Reports

Author

Year

Age

sex

Hgb

M -protein

Other Labs

Underlying disease

Therapy

Long term Outcome

Abbreviations: ACLA: anti cardiolipin antibody; AN A: antineutrophil antibodies; CLL: chronic lymphocytic leukemia; ESR: erythrocyte sedimentation rate; F: female; Hgb: hemoglobin; LDH: lactate dehydrogenase; M: Male; NR: not reported; OSH: outside hospital; Plt: platelets; ppx: prophylactic; T-bili: total bilirubin; TD: transfusion dependent; WBC: white blood cell count.

Gorius [1]

1973

7.75 (Transfusion dependent)

Serum iron 105, TIBC 240, T bili 1.3, Coombs negative,
Retic count 0.5%, Negative HAM’s test, no agglutination with anti-i.

Nagao [3]

1976

Hematocrit 24%, Plt 263 × 10⁴/cm², WBC 5,900/cm², retic count < 1‰

Cramer [4]

1999

Reticulocyte count reduced; nl serum iron;

Small lymphocytic lymphoma

Chlorambucil → no response; corticosteroids → decreased transfusion requirements, anemia recurred with taper; multiagent chemotherapy → hgb stabilized

Died from Richter transformation after 10 years.

5.5

IgG, kappa 3 g/L

Iron 35, ferritin 175, LDH 667, bilirubin 9.3, haptoglobin markedly reduced, Retic count 20 × 10⁹/L Coombs positive for complement, lupus anticoagulant present, ACLA positive

Sjogren’s syndrome

Corticosteroids with resolution of anemia, but relapse with withdrawal of steroids

7.9

IgG, kappa (1.7 g/L)

WBC 18.7 with 76% lymphs,

CLL

6 cycles low dose CHOP, ppx IVIG, 1 year chlorambucil → remission (hgb 12.7); relapsed 6 months later with hgb 6.9; fludarabine 6 mo later with transient incomplete remission

Died from septic cellulitis 3 years later

Bacher [6]

2004

IgG, kappa (3.3 g/dL)

ESR 75, ANA neg, AMA neg, C4 < 0.01, C3 0.52 (low), IgG 35.4 (elevated),
No signs of hemolysis, Retic count 15‰.

None

None (In good clinical condition)

Liapis [7]

2008

4.9

IgG, kappa (1.5 g/L)

Reticulocyte count 1.5 × 10⁹/L; ferritin, B12, and folate nl.

Small lymphocytic lymphoma

Therapy (not specified) of small lymphoctic lymphoma lead to resolution

Zaninoni [5]

2010

Reduced reticulocytes (20 × 10⁹/L), unconjugated bili 1.8, LDH 550,
haptoglobin < 20, DAT negative

Steroids and cyclosporine “with some response” at OSH; Repeat prednisone with clinical response. Relapsed 2 years later and again treated with prednisone taper with good response.

Papakonstantinou [8]

2010

8.1 (Transfusion dependent)

None

WBC 2.8 (29% PMN, 53% lymphocytes), retic count 105.2 × 10⁹ L; LDH 355, IgA undetectable, CD19+ B-lymphocytes reduced (21 × 10⁶/L); no monoclonal B-cell population detected. Serum IgG, IgM, haptoglobin, vitamin B12, folic acid, ferritin, and B2-microglobulin were normal. Coombs negative.

None

Prednisolone with no response, IVIG with no response; complete remission with rituximab

Response maintained at 30 months.