Table 2 Clinical characteristics, neuroimaging findings, treatment strategies, and outcomes of the four cases with primary central nervous system post-transplantation lymphoproliferative disorder (PCNS-PTLD) following hematopoietic stem cell transplant in patients with β-thalassemia from our case series
Characteristic | Case 1 | Case 2 | Case 3 | Case 4 |
|---|---|---|---|---|
Age, y | 5 | 8 | 8 | 4 |
Sex | M | M | F | M |
Brain symptom | Headache, vomiting, seizures | Headache, vomiting, convulsions, loss of speech, and rigidity of his right limb | Appearance of weakness of her lower limbs | Convulsions, lethargy, unresponsiveness, limb weakness, coma |
CT | Slight hyperdensity lesions in right cerebellar hemisphere, left occipital lobe, and pontine brain and frontal-parietal, with hyperdensity hemorrhage in the left frontal lobe | Isodensity multiple nodular lesions in the frontal lobe, parietal lobe, occipital lobe, basal ganglia, cerebellar hemispheres, cerebellar vermis, and corpus callosum and patchy hyperdense hemorrhage were found in the left occipital lobe lesion | None | None |
MRI | Lesions in right cerebellar hemisphere, left occipital lobe, both frontal lobes, parietal lobes, and pontine lobes were hypointense on T1WI and heterogeneous hyper- and hypointense on T2WI. The parietal and frontal lesions on both sides and left temporal lesion showed heterogeneous signal on T1WI, heterogeneous signal on T2WI, and heterogeneous signal on T2-FLAIR images | The lesions in the frontal lobe, parietal lobe, occipital lobe, basal ganglia, cerebellar hemispheres, cerebellar vermis, and corpus callosum were hypointense on T1WI, heterogeneous hyper- and hypointense on T2WI, heterogeneous hyper- and hypointense on T2-FLAIR, hyper- and hypointense on DWI, and slightly hyperintense on ADC, and slightly hyperintense ring-shaped capsule was around some of the larger lesions, and nodular heterogeneous enhancement on enhancement scan, with scaly edema around them | The lesion in the midbrain and pontine brain was hypointense on T1WI, heterogeneous hyper- and hypointense signal on T2WI, and heterogeneous hypointense on T2-FLAIR and ring-shaped apparent uneven enhancement on the enhancement scan | Lesions in the cerebral hemispheres, cerebellar hemispheres, pons, and midbrain were hypointense on T1WI and heterogeneous hyper- and hypointense on T2WI and T2-FLAIR. Some larger lesions showed a slightly hyperintense ring capsule, and the lesions showed nodular light to moderate uneven enhancement on enhanced scan |
CSF | ||||
Protein, mg/L (150.00–450.00) | In normal range | 2020.30 | 316.6 | 943.80 |
Glucose, mmol/L (2.80–4.20) | In normal range | 2.58 | 2.75 | 2.60 |
Chlorine, mmol/L (120.00–132.00) | In normal range | None | 118.5 | 115.90 |
NGS | None | CMV was detected with four reads, and EBV was detected with three reads in CSF samples | None | None |
Histology | Monomorphic, diffuse large B cell. EBV infection-associated post-transplant lymphoproliferative disorder | Post-transplant lymphoproliferative disorder (early lesion: plasmacytic hyperplasia) | None | Monomorphic B-PTLD-EBV-positive diffuse large B-cell lymphoma (post-germinal center subtype) |
Treatment | Craniotomy and medication with rituximab | Craniotomy and medication with rituximab | Medication with methylprednisolone, gammaglobulin, and mannitol. A drainage tube was placed in the ventricle | Craniotomy, and medication with combination of rituximab and MTX |
Outcome | Died | Died | Died | Not known |