J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

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

Journal website https://www.journalmc.org

Case Report

Volume 14, Number 5, May 2023, pages 179-183

A Diagnostic Dilemma: Adult-Onset Still’s Disease With Secondary Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome?

**Table 1.** Patient’s Laboratory Results From Day One to Seven Weeks Follow-Up
Analyte	Day 1	Day 2	Day 3	Day 4	Day 9	Day 10	Day 11	Two weeks later	Five weeks later	Seven weeks later	Reference values
WBCs: white blood cells; AST: aspartate aminotransferase; ALT: alanine transaminase; ALP: alkaline phosphatase; CRP: C-reactive protein; hsCRP: high-sensitivity C-reactive protein; ESR: erythrocyte sedimentation rate.
Hemoglobin (g/dL)	12.3	9.9	10.2	10.2	8.0	7.8	8.2	11.5	11.4	11.6	14 - 17
WBCs count (/µL)	10.7	8.4	8.7	7.7	21.3	19.5	19.5	16.3	6.0	6.1	400 - 10,000
AST (U/L)	66	75	164	304	216	181	895	251	94	34	0 - 35
ALT (U/L)	75	78	167	491	481	518	683	172	86	89	0 - 35
ALP (U/L)	84	94	123	141	273	267	243	211	168	73	36 - 92
CRP (mg/dL)		13.8			17.3	33.2	58.2	3.0	0.7	13.2	0 - 0.8
hsCRP					432.3			> 10.0
ESR (mm/h)		29			56	50	87	20	10	16	0 - 15
D-dimer			1,520
Ferritin (ng/mL)		376			1,289	1,863	14,549	1,593	331	98	15 - 200
Fibrinogen (mg/dL)						140					150 - 350
Triglycerides (mg/dL)					86						≤ 150

Table 1. Patient’s Laboratory Results From Day One to Seven Weeks Follow-Up

Analyte

Day 1

Day 2

Day 3

Day 4

Day 9

Day 10

Day 11

Two weeks later

Five weeks later

Seven weeks later

Reference values

WBCs: white blood cells; AST: aspartate aminotransferase; ALT: alanine transaminase; ALP: alkaline phosphatase; CRP: C-reactive protein; hsCRP: high-sensitivity C-reactive protein; ESR: erythrocyte sedimentation rate.

Hemoglobin (g/dL)

12.3

9.9

10.2

8.0

7.8

8.2

11.5

11.4

11.6

14 - 17

WBCs count (/µL)

10.7

8.4

8.7

7.7

21.3

19.5

16.3

6.0

6.1

400 - 10,000

AST (U/L)

164

304

216

181

895

251

0 - 35

ALT (U/L)

167

491

481

518

683

172

0 - 35

ALP (U/L)

123

141

273

267

243

211

168

36 - 92

CRP (mg/dL)

13.8

17.3

33.2

58.2

3.0

0.7

13.2

0 - 0.8

hsCRP

432.3

> 10.0

ESR (mm/h)

0 - 15

D-dimer

1,520

Ferritin (ng/mL)

376

1,289

1,863

14,549

1,593

331

15 - 200

Fibrinogen (mg/dL)

140

150 - 350

Triglycerides (mg/dL)

≤ 150

**Table 2.** Autoimmune Workup Result
Analyte	Result	Reference value
pANCA: perinuclear antineutrophil cytoplasmic antibodies; cANCA: cytoplasmic antineutrophil cytoplasmic antibodies; CCP: cyclic citrullinated peptides; IL: interleukin; IgG: immunoglobulin G; LKM: liver kidney microsomal; SCL: scleroderma; IgM: immunoglobulin M; HLA: human leukocyte antigen; IgA: immunoglobulin A; tTG: tissue transglutaminase; AI: antibody index.
Antinuclear antibody	Negative
Rheumatoid factor	Negative
pANCA	Negative
cANCA	Negative
Anti-CCP	Negative
IL-2 receptor α (CD25), soluble Il2 Recep SerPl-mCnc	2,473	532 - 1,891 pg/mL
Mitochondrial M2 antibody (IgG)	Negative
LKM-1 antibody (IgG)	Negative
Actin (smooth muscle) antibody IgG	Negative
SCL-70 antibody	Negative
Centromere antibody	< 1.0	AI < 1.0
Cardiolipin IgG	< 1.6	0.0 - 19.9 GPL U/mL
Cardiolipin IgM	0.6	0.0 - 19.9 MPL U/mL
Tryptase	1.8	< 11.0 µg/L
HLA B27	Negative
Deamidated gliadin antibody, IgA	Negative
tTG antibody, IgA	Negative

Table 2. Autoimmune Workup Result

Analyte

Result

Reference value

pANCA: perinuclear antineutrophil cytoplasmic antibodies; cANCA: cytoplasmic antineutrophil cytoplasmic antibodies; CCP: cyclic citrullinated peptides; IL: interleukin; IgG: immunoglobulin G; LKM: liver kidney microsomal; SCL: scleroderma; IgM: immunoglobulin M; HLA: human leukocyte antigen; IgA: immunoglobulin A; tTG: tissue transglutaminase; AI: antibody index.

Antinuclear antibody

Negative

Rheumatoid factor

Negative

pANCA

Negative

cANCA

Negative

Anti-CCP

Negative

IL-2 receptor α (CD25), soluble Il2 Recep SerPl-mCnc

2,473

532 - 1,891 pg/mL

Mitochondrial M2 antibody (IgG)

Negative

LKM-1 antibody (IgG)

Negative

Actin (smooth muscle) antibody IgG

Negative

SCL-70 antibody

Negative

Centromere antibody

< 1.0

AI < 1.0

Cardiolipin IgG

< 1.6

0.0 - 19.9 GPL U/mL

Cardiolipin IgM

0.6

0.0 - 19.9 MPL U/mL

Tryptase

1.8

< 11.0 µg/L

HLA B27

Negative

Deamidated gliadin antibody, IgA

Negative

tTG antibody, IgA

Negative

**Table 3.** Diagnostic Guidelines for HLH
HLH: hemophagocytic lymphohistiocytosis; NK: natural killer; sIL-2R: soluble interleukin 2 receptor.
1. Fever
2. Splenomegaly
3. Cytopenias affecting ≥ 2 lineages
a. Hemoglobin < 9 g/dL
b. Platelets < 100 × 10⁹/L
c. Neutrophils < 1.0 × 10⁹/L
4. Hypertriglyceridemia and/or hypofibrinogenemia
a. Triglycerides ≥ 265 mg/dL
b. Fibrinogen ≤ 150 mg/dL
5. Hemophagocytosis in bone marrow, spleen, or lymph nodes
6. Low or absent NK cell activity
7. Ferritin ≥ 500 µg/L
8. sCD25 (i.e., sIL-2R) ≥ 2,400 U/mL

Table 3. Diagnostic Guidelines for HLH

HLH: hemophagocytic lymphohistiocytosis; NK: natural killer; sIL-2R: soluble interleukin 2 receptor.

1. Fever

2. Splenomegaly

3. Cytopenias affecting ≥ 2 lineages

a. Hemoglobin < 9 g/dL

b. Platelets < 100 × 10⁹/L

c. Neutrophils < 1.0 × 10⁹/L

4. Hypertriglyceridemia and/or hypofibrinogenemia

a. Triglycerides ≥ 265 mg/dL

b. Fibrinogen ≤ 150 mg/dL

5. Hemophagocytosis in bone marrow, spleen, or lymph nodes

6. Low or absent NK cell activity

7. Ferritin ≥ 500 µg/L

8. sCD25 (i.e., sIL-2R) ≥ 2,400 U/mL