Table I.
Patients with symptoms consistent with leucostasis* | Asymptomatic patients | ||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Patient no. | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 | 13 | 14 | 15 |
Age, years | 15 | 5 months | 8 | 13 | 5 | 7 | 8 | 3 | 7 | 16 | 18 | 3 | 14 | 6 months | 2 |
Imaging | NA | Brain MR - CSF prominence | Brain MR - thalamic infarct | Brain MR - normal | Normal | Normal | Normal | Normal | Normal | Normal | Normal | Normal | CXR - large anterior mass | Normal | Normal |
Symptoms and physical examination findings | Haemoptysis, respiratory failure** | Bilateral papilledema, chloroma of right orbit | Dyspnea, cough, tachnypnea | Blurry vision in left eye with decreased visual acuity, bilateral papilledema | Normal | Normal | Splenomegaly | Normal | Normal | Normal | Normal | Normal | Normal | Normal | Normal |
Sex, M/F | Male | Female | Male | Female | Male | Male | Male | Female | Male | Female | Female | Female | Male | Female | Female |
ALL subtype | Pre-B (refractory, relapsed) | Pre-B (CALLA negative) | T-cell precursor | Pre-B (CALLA negative) | Pre-B | Pre-B | T-cell precursor | Pre-B | T-cell precursor | Pre-B | Pre-B | Pre-B | T-cell precursor | Pre-B | Pre-B |
Cytogenetics | t(9;22) (q34;qll) | t(4;11) (q21;q23) | Normal | t(4;11) (q21;q23) | Normal | Normal | t(11;14) (pl3;ql) | Normal | -Y, −9p | Normal | Normal | Hyperploidy | XYY, t(2;13) | t(4;ll) (q21;q23) | Hyperploidy |
WBC × 109/l | 33·3 | 420·5 | 453·0 | 169·0 | 142·4 | 256·3 | 476·3 | 189·4 | 221·0 | 2·1 | 55·0 | 51·8 | 225.0 | 14.4 | 94.1 |
Blasts × 109/l | 32·0 | 395·3 | 317·1 | 150·4 | 110·7 | 230·2 | 433·2 | 158·6 | 179·01 | 0·4 | 52·8 | 36·77 | 209·25 | 11·52 | 81·87 |
Hb (g/l) | 93 | 67 | 113 | 40 | 56 | 52 | 97 | 82 | 98 | 85 | 86 | 81 | 53 | 107 | 97 |
Platelets × 109/l | 26 | 21 | 9 | 7 | 125 | 11 | 45 | 65 | 44 | 78·0 | 39 | 37 | 37 | 50 | 37 |
Uric acid (μmol/l) | 53·5 | 374·7 | 398·5 | 339·0 | 463·9 | 190·3 | 398·5 | 410·4 | 547·2 | 422·3 | 446·1 | 130·9 | 606·7 | 172·5 | 565·1 |
Lactate Dehydrogenase (IU/l) | NA | 1619 | >2700 | 1266 | 825 | 1225 | 2544 | 543 | 7000 | 434 | 1069 | 407 | 707 | 317 | 1250 |
NA, not available; MR, magnetic resonance; CSF, cerebrospinal fluid; ALL, acute lymphoblastic leukaemia; WBC, white blood cells.
Although leucostasis may occur in other organ systems as well, only haematological, neurological, and/or respiratory dysfunction were observed in our patient sample. In addition, no evidence of serious bacterial infection/sepsis (all patients were afebrile at the time of the blood draw) or renal dysfunction due to uric acid nephropathy and tumor lysis syndrome was seen in any of the patients studied.
Of note, patient 1 was diagnosed with aspergillous pneumonia (diagnosed via broncho-alveolar lavage) 6 months prior to his death. He was successfully treated with caspofungin and voriconazole, which he received continuously until his death. During the last 2 weeks of his life, he did not experience any respiratory symptoms or fever until the day of his death. Serial chest radiographs revealed no abnormality during that time. The only detectable change in his clinical status, aside from increase in leukaemia cell stiffness, was an increasing number of circulating leukaemia cells during his last week (leukaemia cell concentration during his last 3 d of life: 10·92, 15·57, 32·0 × 109 cells/l respectively). On the day he died, he remained afebrile but developed progressively worsening respiratory failure over several hours and haemoptysis in the last hour of life. The correlation between cell concentration and cell stiffness was not statistically significant (P = 0.23), and the leukaemia cell concentration did not reach levels consistent with hyperleucocytosis.