Severe autoimmune hemolytic anemia complicating hereditary spherocytosis treated successfully with glucocorticoids and cyclosporine: a case report

Figures & data

Table 1. The patient's laboratory indices and clinical characteristics.

Clinical characteristics	Jan. 30th	Feb. 5th	Feb. 14th	Feb. 21st	Mar. 1st	Reference values*
WBC (×10^9/L)	2.93	5.83	6.90	8.42	12.23	3.50- 9.50
RBC (×10^12/L)	0.83	1.24	0.90	2.11	2.69	3.80- 5.10
Hb (g/L)	27.00	36.00	26.00	63.00	80.00	115.00- 150.00
MCV (fL)	91.60	89.50	91.10	93.40	90.30	82.00- 100.00
MCH (pg)	32.50	29.00	28.90	29.90	29.70	27.00- 34.00
MCHC (g/L)	355.00	324.00	317.00	320.00	329.00	316.00- 354.00
Hematocrit level (%)	7.60	11.10	8.20	19.70	24.30	35.00- 45.00
RDW %	22.80	16.80	23.80	21.30	21.10	11.00- 15.00
Ret (%)	6.41	10.23	13.96	15.57	9.77	0.50- 1.50
Platelets (×10^9/L)	80.00	106.00	118.00	142.00	149.00	100.00- 350.00
ALT (IU/L)	9.00	5.00	13.00	7.00	23.00	0- 40.00
AST (IU/L)	28.00	4.00	13.00	8.00	16.00	0- 42.00
TBIL (µmol/L)	134.68	66.61	62.78	122.22	92.41	5.00- 21.00
DBIL (µmol/L)	29.74	18.97	19.31	16.08	20.04	0- 7.00
LDH (IU/L)	609.00	NT	189.00	181.00	262.00	100.00- 250.00
Irregular Ab	+	+	+	+	+	negative
DAT for anti-IgG	2+	NT	NT	NT	0	negative
DAT for anti-C3d	1+	NT	NT	NT	0	negative

Note: WBC, white blood cells; RBC, red blood cells; Hb, hemoglobin; MCV, mean corpuscular volume; MCH, mean corpuscular hemoglobin; MCHC, mean corpuscular hemoglobin concentration; RDW, red blood cell volume distribution width; Ret, reticulocytes; ALT, alanine aminotransferase; AST, aspartate aminotransferase; TBIL, total bilirubin; DBIL, direct bilirubin; LDH, lactate dehydrogenase; NT, not tested; Ab, antibodies; DAT, direct anti-human globulin test; IgG, immunoglobulin G; 0, no agglutination.

*Reference values are affected by many variables, including the patient population and laboratory methods used. The ranges used at the China-Japan Friendship Hospital are for adults who are not pregnant and have no medical condition that could affect the results. They are thus not appropriate for all patients.

Figure 1. Timeline of the therapeutic intervention.

Figure 2. Pedigree of the hereditary spherocytosis (HS) family. Males and females are represented by squares and circles, respectively. The blank symbol represents the wild type and the half-filled symbols indicate heterozygosity. The index case is marked by an arrow.

Table 2. Information on the patient's SPTB mutation.

Gene	Location	cDNA change	Protein change	Status	Type	Inheritance	ACMG Classification
SPTB	Exon21	c.4538_4539 del.TG	p.V1513Afs*13	Het	Frameshift	Inherited	5-P

Abbreviations: c, variation at the cDNA level; p, variation at the protein level; Het, heterogenous; 5-P, pathogenic.

Transcripts SPTB: NM_000347.

Note: The sequence variants were interpreted according to the recommendations of the American College of Medical Genetics and Genomics and described using standard terminology.

Data availability statement

The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.