World J Oncol

World Journal of Oncology, ISSN 1920-4531 print, 1920-454X online, Open Access

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

Journal website http://www.wjon.org

Case Report

Volume 9, Number 4, August 2018, pages 123-127

A Case of Recurrent Pregnancy-Induced Adult Onset Familial Hemophagocytic Lymphohistiocytosis

Figure 1. Morphologic evidence of hemophagocytosis: bone marrow pathology pictures. (a) Macrophage with intracytoplasmic nuclear debris, red blood cells, and platelets (hemophagocytosis) (BM aspirate smear, Wright Giemsa stain, 1,000 × magnification). (b) Macrophage with numerous intracytoplasmic red blood cells (erythrophagocytosis) (BM aspirate smear, Wright Giemsa stain, 1,000 × magnification). (c) Extensive macrophage infiltration of bone marrow, highlighting scattered hemophagocytic cells (inset, 1,000 × magnification) (BM trephine core biopsy, CD68 immunohistochemical stain).

**Table 1.** Labs Upon Presentation, After Treatment, Outpatient Clinic 2 Months Prior to Admission and Upon Re-Presentation
Labs	Diagnosis	Discharge	Outpatient	Relapse	Reference range
Β-hCG: beta human chorionic gonadotropin; ANC: absolute neutrophil count; LDH: lactate dehydrogenase; AST: aspartate aminotransferase; ALT: alanine aminotransferase.
Β-hCG (mIU/mL)	112	3		53,630	0 - 5
Hemoglobin (g/L)	8.5	7.9	12.3	9.0	12.0 - 14.6
ANC (10⁹/L)	1.7	1.0	1.2	1.1	1.8 - 8.0
Platelets (10⁹/L)	29	127	134	33	160 - 360
Ferritin (ng/mL)	4,122	870	162	> 8,000	10 - 150
Fibrinogen (mg/dL)	< 60	421	276	93	239 - 439
Triglycerides (mg/dL)	224	199			≤ 149
LDH (U/L)	2,326	182	164	2,288	90 - 220
AST (U/L)	748	30	28	504	10 - 40
ALT (U/L)	297	33	27	153	5 - 40
Bilirubin (mg/dL)	6.2	0.3		4.6	0.0 - 1.0

Table 1. Labs Upon Presentation, After Treatment, Outpatient Clinic 2 Months Prior to Admission and Upon Re-Presentation

Labs

Diagnosis

Discharge

Outpatient

Relapse

Reference range

Β-hCG: beta human chorionic gonadotropin; ANC: absolute neutrophil count; LDH: lactate dehydrogenase; AST: aspartate aminotransferase; ALT: alanine aminotransferase.

Β-hCG (mIU/mL)

112

53,630

0 - 5

Hemoglobin (g/L)

8.5

7.9

12.3

9.0

12.0 - 14.6

ANC (10⁹/L)

1.7

1.0

1.2

1.1

1.8 - 8.0

Platelets (10⁹/L)

127

134

160 - 360

Ferritin (ng/mL)

4,122

870

162

> 8,000

10 - 150

Fibrinogen (mg/dL)

< 60

421

276

239 - 439

Triglycerides (mg/dL)

224

199

≤ 149

LDH (U/L)

2,326

182

164

2,288

90 - 220

AST (U/L)

748

504

10 - 40

ALT (U/L)

297

153

5 - 40

Bilirubin (mg/dL)

6.2

0.3

4.6

0.0 - 1.0

**Table 2.** Modified HLH-94 Protocol
Agents	Dose	Route	Days of treatment
Etoposide	150 mg/m² (240 mg)	Intravenous	D1, 4, 8 and 11
Etoposide	112 mg/m² (220 mg)	Intravenous	D15, 22, 29, 36, 43, and 50
Dexamethasone	10 mg/m² (15 mg)	Oral	D1 - 14
Dexamethasone	5 mg/m² (8 mg)	Oral	D15 - 28
Dexamethasone	2.5 mg/m² (4 mg)	Oral	D29 - 42
Dexamethasone	1.25 mg/m² (2 mg)	Oral	D43 - 49

Table 2. Modified HLH-94 Protocol

Agents

Dose

Route

Days of treatment

Etoposide

150 mg/m² (240 mg)

Intravenous

D1, 4, 8 and 11

Etoposide

112 mg/m² (220 mg)

Intravenous

D15, 22, 29, 36, 43, and 50

Dexamethasone

10 mg/m² (15 mg)

Oral

D1 - 14

Dexamethasone

5 mg/m² (8 mg)

Oral

D15 - 28

Dexamethasone

2.5 mg/m² (4 mg)

Oral

D29 - 42

Dexamethasone

1.25 mg/m² (2 mg)

Oral

D43 - 49