Int J Clin Pediatr

International Journal of Clinical Pediatrics, ISSN 1927-1255 print, 1927-1263 online, Open Access

Article copyright, the authors; Journal compilation copyright, Int J Clin Pediatr and Elmer Press Inc

Journal website http://www.theijcp.org

Case Report

Volume 8, Number 1, August 2019, pages 8-11

Rapid Response to Low-Dose Rituximab Following Development of Severe Hemophagocytic Lymphohistiocytosis Due to Epstein-Barr Virus Infection

Figure 1. Microscopic findings of bone marrow specimens on day 1. Bone marrow showed monocytic infiltration with a background of hypocellular lesion (May-Giemsa stain, × 400). Lymphocytes account for 12% of total nucleated cells, comprising the following subsets: CD3, 93%; CD4, 36%; CD8, 55%; CD19, 2%; CD56, 4%.

Figure 2. Response to prednisolone, cyclosporine and rituximab. The patient did not respond to first-line therapy with prednisolone. A high level of EBV-DNA was identified on day 4. After the first course of rituximab that was administered on day 5, disease activity rapidly declined. CsA: cyclosporine; DIC: disseminated intravascular coagulation; EBV: Epstein-Barr virus; Plt: platelets; PSL: prednisolone; WBC: white blood cells.

**Table 1.** Laboratory Data
	Normal range	On admission	Outpatient clinic
Alb: albumin; ALT: alanine aminotransferase; ANA: anti-nuclear antibody; AST: aspartate aminotransferase; AT: antithrombin; Cr: creatinine; CRP: C-reactive protein; FDP: fibrin and fibrinogen degradation product; Hb: hemoglobin; LDH: lactate dehydrogenase; PIC: plasmin-α2-plasmin inhibitor complex; Plt: platelets; PT: prothrombin time ; sIL2R: soluble interleukin 2 receptor; TAT: thrombin-antithrombin complex; TB: total bilirubin; TP: total protein; UN: urea nitrogen; WBC: white blood cells; Nd: not determined.
WBC (10⁹/L)	3.3 - 8.6	1	1	0.3	1.4	1.9	2.4	3.6	8.4	5.9	4.5	3.1	3.4	3.6	2.9	4.4	3.8
Neutrophil (%)		64	95	94	96	96.5	Nd	95	93	72	68	72	68	74	71	76	69
Lymphocyte (%)		28	4	3	3	3	Nd	2	2	18	13	16	13	20	23	20	26
Monocyte (%)		7	1	3	1	0.5	Nd	4	5	10	19	12	16	5	4	4	4
Hb (g/L)	116 - 148	137	126	107	104	113	102	97	98	95	99	111	113	124	127	135	132
Reticulocyte (‰)	4 - 20	7	9	5	3	3	2	2	3	3	45	103	51	16	10	10	14
Plt (10⁹/L)	158 - 348	48	58	36	33	48	33	47	77	139	235	310	346	178	171	198	195
TB (mg/L)	3 - 12	9.6	7.4	6	10.8	13	8.9	6.6	7.7	7.5	8.7	9.1	10.5	9.9	5.7	6.3	6.3
AST (U/L)	13 - 30	92	143	227	272	247	218	125	84	73	45	40	24	24	22	23	29
ALT (U/L)	10 - 42	70	63	102	154	171	165	126	107	132	106	105	55	21	15	34	38
LDH (U/L)	124 - 222	692	825	1,046	1,297	1,245	901	677	497	420	385	317	238	175	130	156	144
UN (mg/L)	80 - 190	74	79	85	83	103	105	148	134	128	133	210	199	86	122	106	84
Cr (mg/L)	5 - 9	8.1	8.2	6.1	4.7	5.2	5.2	5	5.1	4.6	4.7	5.6	5.3	5.8	5.6	6.4	6
TP (g/L)	65 - 80	63	51	45	43	46	47	51	58	59	67	71	69	73	67	71	67
Alb (g/L)	38 - 53	36	29	24	23	26	27	30	39	39	44	48	48	51	47	49	46
Ferritin (µg/L)	20 - 120	1,921	Nd	Nd	Nd	8,479.9	Nd	3,849.6	Nd	1,842	Nd	307.8	Nd	58	9	6.9	11
sIL2-R (U/mL)	145 - 519	9,090	Nd	Nd	Nd	14,400	Nd	Nd	Nd	4,880	Nd	1,680	Nd	434	182	261	248
CRP (mg/L)	0 - 2	80.3	66.1	40.1	31.8	18.9	10.3	6.9	4.4	2.5	1.2	< 1	< 1	< 1	< 1	< 1	< 1
ANA	(-)		Negative
PT ratio	0.9 - 1.1	1.26	1.13	1.18	1.09	0.99	0.94	0.9	0.86	0.9	0.9	0.91	0.93	1	1.02	Nd	Nd
Fibrinogen (g/L)	1.5 - 4.0	3.34	2.77	1.53	1.44	1.22	1.27	1.13	1.26	1.21	1.23	1.46	1.5	286	2.23	Nd	Nd
FDP (mg/L)	< 5	13.9	20.8	104.6	67.8	55.3	30.5	18	11.1	7.1	< 5	< 5	< 5	< 5	< 5	Nd	Nd
D-dimer (mg/L)	< 1	13	20	88.5	48.3	45	28.1	15.9	9	5.5	2.7	1.8	< 1	< 1	< 1	Nd	Nd
AT (%)	70 - 130	97	87	82	89	109	120	128	135	144	144	> 150	> 150	140	132	Nd	Nd
TAT (µg/L)	< 3	26.8	59	80.2	52	38.3	21.4	12.4	2.9	2.7	2.9	1.5	1.5	< 1	Nd	Nd	Nd
PIC (mg/L)	< 1	2.7	3.1	Nd	5.8	6.4	6.3	4.2	3	2	1.4	1.5	< 1	< 1	Nd	Nd	Nd
Urinalysis
Protein	(-)	+/-	1+	1+	1+	1+	+/-	+/-	-	Nd	-	-	-	Nd	Nd	Nd	Nd
Blood	(-)	+/-	3+	2+	1+	-	-	-	-	Nd	-	-	-	Nd	Nd	Nd	Nd
Sugar	(-)	-	-	-	-	-	-	-	-	Nd	-	-	-	Nd	Nd	Nd	Nd
Blood culture		Negative

Table 1. Laboratory Data

Normal range

On admission

Outpatient clinic

day 1

day 2

day 4

day 5

day 6

day 7

day 8

day 9

day 11

day 13

day 15

day 18

day 30

day 56

day 126

day 195

Alb: albumin; ALT: alanine aminotransferase; ANA: anti-nuclear antibody; AST: aspartate aminotransferase; AT: antithrombin; Cr: creatinine; CRP: C-reactive protein; FDP: fibrin and fibrinogen degradation product; Hb: hemoglobin; LDH: lactate dehydrogenase; PIC: plasmin-α2-plasmin inhibitor complex; Plt: platelets; PT: prothrombin time ; sIL2R: soluble interleukin 2 receptor; TAT: thrombin-antithrombin complex; TB: total bilirubin; TP: total protein; UN: urea nitrogen; WBC: white blood cells; Nd: not determined.

WBC (10⁹/L)

3.3 - 8.6

0.3

1.4

1.9

2.4

3.6

8.4

5.9

4.5

3.1

3.4

3.6

2.9

4.4

3.8

Neutrophil (%)

96.5

Lymphocyte (%)

Monocyte (%)

0.5

Hb (g/L)

116 - 148

137

126

107

104

113

102

111

113

124

127

135

132

Reticulocyte (‰)

4 - 20

103

Plt (10⁹/L)

158 - 348

139

235

310

346

178

171

198

195

TB (mg/L)

3 - 12

9.6

7.4

10.8

8.9

6.6

7.7

7.5

8.7

9.1

10.5

9.9

5.7

6.3

AST (U/L)

13 - 30

143

227

272

247

218

125

ALT (U/L)

10 - 42

102

154

171

165

126

107

132

106

105

LDH (U/L)

124 - 222

692

825

1,046

1,297

1,245

901

677

497

420

385

317

238

175

130

156

144

UN (mg/L)

80 - 190

103

105

148

134

128

133

210

199

122

106

Cr (mg/L)

5 - 9

8.1

8.2

6.1

4.7

5.2

5.1

4.6

4.7

5.6

5.3

5.8

5.6

6.4

TP (g/L)

65 - 80

Alb (g/L)

38 - 53

Ferritin (µg/L)

20 - 120

1,921

8,479.9

3,849.6

1,842

307.8

6.9

sIL2-R (U/mL)

145 - 519

9,090

14,400

4,880

1,680

434

182

261

248

CRP (mg/L)

0 - 2

80.3

66.1

40.1

31.8

18.9

10.3

6.9

4.4

2.5

1.2

< 1

ANA

(-)

Negative

PT ratio

0.9 - 1.1

1.26

1.13

1.18

1.09

0.99

0.94

0.9

0.86

0.9

0.91

0.93

1.02

Fibrinogen (g/L)

1.5 - 4.0

3.34

2.77

1.53

1.44

1.22

1.27

1.13

1.26

1.21

1.23

1.46

1.5

286

2.23

FDP (mg/L)

< 5

13.9

20.8

104.6

67.8

55.3

30.5

11.1

7.1

< 5

D-dimer (mg/L)

< 1

88.5

48.3

28.1

15.9

5.5

2.7

1.8

< 1

AT (%)

70 - 130

109

120

128

135

144

> 150

140

132

TAT (µg/L)

< 3

26.8

80.2

38.3

21.4

12.4

2.9

2.7

2.9

1.5

< 1

PIC (mg/L)

< 1

2.7

3.1

5.8

6.4

6.3

4.2

1.4

1.5

< 1

Urinalysis

Protein

(-)

+/-

Blood

(-)

+/-

Sugar

(-)

Blood culture

Negative