Figure 2
Figure 2. Long-term follow-up after immunosuppression. In patients treated with immunosuppression, we follow for relapse (among responders) and clonal evolution in all patients. A gradual downtrend in blood counts may signify hematologic response, underscoring the important of routine monitoring in this setting. In cases of relapse, we usually reintroduce more immunosuppression in the form of oral cyclosporine and/or a repeat course with rabbit ATG/CsA or alemtuzumab. In those who are unresponsive to more immunosuppression, further management will depend on suitability for HSCT (age, donor availability, comorbidities). When only higher risk HSCT options are available (mismatched unrelated, haploidentical, umbilical cord), we consider nonimmunosuppressive strategies, such as androgens (12-week trial), combination growth factors (G-CSF + Epo for 12 weeks), or experimental therapies. In patients with a very low neutrophil count unresponsive to G-CSF associated with infections, we consider a higher risk HSCT in younger patients. We monitor for clonal evolution by repeated marrow karyotype assessment at 6 and 12 months and then yearly thereafter. After 5 years, we tend to increase the interval between bone marrows. When faced with an abnormal karyotype, such as del13q, trisomy 6, pericentric inversion of chromosome 1;9, del20q, or trisomy 8, we assess for myelodysplasia by looking at blood counts, peripheral smear, and bone marrow morphology. On occasion, these karyotypes may not equate to progression to myelodysplasia and not be detected on repeated marrow examination. In cases where there is worsening blood counts and/or more significant dysplastic changes in the marrow, our approach is to seek transplant options, therapies for myelodysplasia, or a clinical trial. Monosomy 7 is almost never a transient finding and commonly associates to a more rapid progression to myelodysplasia and leukemia. In these cases, our approach is to seek HSCT earlier.

Long-term follow-up after immunosuppression. In patients treated with immunosuppression, we follow for relapse (among responders) and clonal evolution in all patients. A gradual downtrend in blood counts may signify hematologic response, underscoring the important of routine monitoring in this setting. In cases of relapse, we usually reintroduce more immunosuppression in the form of oral cyclosporine and/or a repeat course with rabbit ATG/CsA or alemtuzumab. In those who are unresponsive to more immunosuppression, further management will depend on suitability for HSCT (age, donor availability, comorbidities). When only higher risk HSCT options are available (mismatched unrelated, haploidentical, umbilical cord), we consider nonimmunosuppressive strategies, such as androgens (12-week trial), combination growth factors (G-CSF + Epo for 12 weeks), or experimental therapies. In patients with a very low neutrophil count unresponsive to G-CSF associated with infections, we consider a higher risk HSCT in younger patients. We monitor for clonal evolution by repeated marrow karyotype assessment at 6 and 12 months and then yearly thereafter. After 5 years, we tend to increase the interval between bone marrows. When faced with an abnormal karyotype, such as del13q, trisomy 6, pericentric inversion of chromosome 1;9, del20q, or trisomy 8, we assess for myelodysplasia by looking at blood counts, peripheral smear, and bone marrow morphology. On occasion, these karyotypes may not equate to progression to myelodysplasia and not be detected on repeated marrow examination. In cases where there is worsening blood counts and/or more significant dysplastic changes in the marrow, our approach is to seek transplant options, therapies for myelodysplasia, or a clinical trial. Monosomy 7 is almost never a transient finding and commonly associates to a more rapid progression to myelodysplasia and leukemia. In these cases, our approach is to seek HSCT earlier.

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