Hematologic and other complications post-lung transplant in 3 heterozygous PARN and 2 heterozygous TERT variant carriers
| ID . | Sex . | Gene . | Age at transplant, y . | Lung transplant . | Follow-up, mo . | Immunosuppressants . | Immunosuppressant dose reduction . | CBC pretransplant . | Hematologic complications . | Respiratory complications . | Wound healing complications . | Infectious complications . | CMV status D/R . | CMV reactivation . | Renal complications . | Gastrointestinal complications . | Other complications . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 557 II.1 | M | PARN | 52 | Double | 24 | Basiliximab induction, methylprednisolone, MMF, tacrolimus, leflunomide | Tacrolimus (AKI), leflunomide discontinued (pancytopenia) | WBC 9.5 × 103/μL; ANC 6.93 × 103/μL; Hb 17.7 g/dL; MCV 90.8 fL; Plts 204 × 103/μL | Hypogammaglobulinemia, anemia (∼8 g/dL, requiring RBC transfusions), thrombocytopenia (∼70-130 × 103/μL), neutropenia (∼1 × 103/μL, requiring G-CSF) | Primary graft dysfunction with lung opacity and atelectasis, bronchopleural fistula, multiple air leaks due to anastomosis insufficiency s/p multiple stents, intercostal muscle flap and pericardial wrap, chronic respiratory failure, ventilation dependency | Sternal dehiscence, anastomosis insufficiency | Sepsis (Enterococcus cloacae, Enterococcus faecalis), pulmonary infection (Staphylococcus epidermidis, rhinovirus, Candida famata, Candida guilliermondii, Candida glabrata, RSV, Pseudomonas, adenovirus, Providencia stuartii, Streptococcus agalactiae, Proteus mirabilis), gastrointestinal infection (norovirus) | D+/R– | Yes, no organ manifestation (positive BAL) | Recurrent AKI (max. creatinine 3.6 mg/dL, BUN 114 mg/dL), intermittent hemodialysis | Transaminitis, paralytic ileus | Atrial flutter, severe RV dysfunction, acute DVT, hypothyroidism, moderate encephalopathy, rectus sheath hematoma, prolonged hospitalization for 11 mo |
| 1080 III.1 | M | PARN | 53 | Double | 3 | Methylprednisolone, MMF, tacrolimus | WBC 9.4 × 103/μL; ANC 6.2 × 103/μL; Hb 13.3 g/dL; MCV 99.6 fL; Plts 262 × 103/μL | Primary graft dysfunction, left hemidiaphragm paresis | Pulmonary infection (MSSA, donor-derived) | D+/R– | Transaminitis, delayed gastric emptying | Cardiogenic shock with pulmonary edema, steroid-induced DM, actinic keratosis/seborrheic dermatitis | |||||
| 841 II.1 | M | PARN | 54 | Double | 2 | Basiliximab induction, methylprednisolone, MMF, tacrolimus | WBC 8.5 × 103/μL; ANC 5.65 × 103/μL; Hb 14.3 g/dL; MCV 87 fL; Plts 242 × 103/μL | Bilateral pleural effusion s/p pigtail insertion on left side | Wound dehiscence | D+/R– | Atrial fibrillation, acute DVT, steroid-induced DM | ||||||
| 1020 II.1 | F | TERT | 53 | Double | 39 | Basiliximab induction, methylprednisolone, MMF, tacrolimus, ATG, plasmapheresis, IVIG | MMF (neutropenia) | WBC 5.3 × 103/μL; ANC 3.02 × 103/μL; Hb 12.9 g/dL; MCV 101.6 fL; Plts 201 × 103/μL | Anemia (∼9 g/dL), thrombocytopenia (70-130 × 103/μL), neutropenia (∼1 × 103/μL, requiring G-CSF) | Right pleural effusion s/p VATS-decortication | Pulmonary infection (rhinovirus, parainfluenza), recurrent URI with Pseudomonas and coronavirus | D–/R+ | Atrial fibrillation, supraventricular tachycardia, steroid-induced DM, osteoporosis | ||||
| 669 III.1 | M | TERT | 53 | Double | 12 | Basiliximab induction, methylprednisolone, tacrolimus, azathioprine | Tacrolimus (AKI), azathioprine (neutropenia, anemia) | WBC 7.3 × 103/μL; ANC 5.54 × 103/μL; Hb 13 g/dL; MCV 101.9 fL; Plts 178 × 103/μL | Anemia (∼8 g/dL, requiring RBC transfusions), thrombocytopenia (∼100-150 × 103/μL), neutropenia (∼1.3 × 103/μL) | ARF in the setting of Nocardia sepsis and AKI | Wound dehiscence of chest wall | Pneumonia (Nocardia with possible brain involvement) | D+/R– | AKI (max. creatinine 4.5 mg/dL, BUN 94 mg/dL), intermittent hemodialysis | Transaminitis, elevated bilirubin | Atrial fibrillation, recurrent pericardial effusions, steroid-induced DM |
| ID . | Sex . | Gene . | Age at transplant, y . | Lung transplant . | Follow-up, mo . | Immunosuppressants . | Immunosuppressant dose reduction . | CBC pretransplant . | Hematologic complications . | Respiratory complications . | Wound healing complications . | Infectious complications . | CMV status D/R . | CMV reactivation . | Renal complications . | Gastrointestinal complications . | Other complications . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 557 II.1 | M | PARN | 52 | Double | 24 | Basiliximab induction, methylprednisolone, MMF, tacrolimus, leflunomide | Tacrolimus (AKI), leflunomide discontinued (pancytopenia) | WBC 9.5 × 103/μL; ANC 6.93 × 103/μL; Hb 17.7 g/dL; MCV 90.8 fL; Plts 204 × 103/μL | Hypogammaglobulinemia, anemia (∼8 g/dL, requiring RBC transfusions), thrombocytopenia (∼70-130 × 103/μL), neutropenia (∼1 × 103/μL, requiring G-CSF) | Primary graft dysfunction with lung opacity and atelectasis, bronchopleural fistula, multiple air leaks due to anastomosis insufficiency s/p multiple stents, intercostal muscle flap and pericardial wrap, chronic respiratory failure, ventilation dependency | Sternal dehiscence, anastomosis insufficiency | Sepsis (Enterococcus cloacae, Enterococcus faecalis), pulmonary infection (Staphylococcus epidermidis, rhinovirus, Candida famata, Candida guilliermondii, Candida glabrata, RSV, Pseudomonas, adenovirus, Providencia stuartii, Streptococcus agalactiae, Proteus mirabilis), gastrointestinal infection (norovirus) | D+/R– | Yes, no organ manifestation (positive BAL) | Recurrent AKI (max. creatinine 3.6 mg/dL, BUN 114 mg/dL), intermittent hemodialysis | Transaminitis, paralytic ileus | Atrial flutter, severe RV dysfunction, acute DVT, hypothyroidism, moderate encephalopathy, rectus sheath hematoma, prolonged hospitalization for 11 mo |
| 1080 III.1 | M | PARN | 53 | Double | 3 | Methylprednisolone, MMF, tacrolimus | WBC 9.4 × 103/μL; ANC 6.2 × 103/μL; Hb 13.3 g/dL; MCV 99.6 fL; Plts 262 × 103/μL | Primary graft dysfunction, left hemidiaphragm paresis | Pulmonary infection (MSSA, donor-derived) | D+/R– | Transaminitis, delayed gastric emptying | Cardiogenic shock with pulmonary edema, steroid-induced DM, actinic keratosis/seborrheic dermatitis | |||||
| 841 II.1 | M | PARN | 54 | Double | 2 | Basiliximab induction, methylprednisolone, MMF, tacrolimus | WBC 8.5 × 103/μL; ANC 5.65 × 103/μL; Hb 14.3 g/dL; MCV 87 fL; Plts 242 × 103/μL | Bilateral pleural effusion s/p pigtail insertion on left side | Wound dehiscence | D+/R– | Atrial fibrillation, acute DVT, steroid-induced DM | ||||||
| 1020 II.1 | F | TERT | 53 | Double | 39 | Basiliximab induction, methylprednisolone, MMF, tacrolimus, ATG, plasmapheresis, IVIG | MMF (neutropenia) | WBC 5.3 × 103/μL; ANC 3.02 × 103/μL; Hb 12.9 g/dL; MCV 101.6 fL; Plts 201 × 103/μL | Anemia (∼9 g/dL), thrombocytopenia (70-130 × 103/μL), neutropenia (∼1 × 103/μL, requiring G-CSF) | Right pleural effusion s/p VATS-decortication | Pulmonary infection (rhinovirus, parainfluenza), recurrent URI with Pseudomonas and coronavirus | D–/R+ | Atrial fibrillation, supraventricular tachycardia, steroid-induced DM, osteoporosis | ||||
| 669 III.1 | M | TERT | 53 | Double | 12 | Basiliximab induction, methylprednisolone, tacrolimus, azathioprine | Tacrolimus (AKI), azathioprine (neutropenia, anemia) | WBC 7.3 × 103/μL; ANC 5.54 × 103/μL; Hb 13 g/dL; MCV 101.9 fL; Plts 178 × 103/μL | Anemia (∼8 g/dL, requiring RBC transfusions), thrombocytopenia (∼100-150 × 103/μL), neutropenia (∼1.3 × 103/μL) | ARF in the setting of Nocardia sepsis and AKI | Wound dehiscence of chest wall | Pneumonia (Nocardia with possible brain involvement) | D+/R– | AKI (max. creatinine 4.5 mg/dL, BUN 94 mg/dL), intermittent hemodialysis | Transaminitis, elevated bilirubin | Atrial fibrillation, recurrent pericardial effusions, steroid-induced DM |
ANC, absolute neutrophil count; AKI, acute kidney failure; ARF, acute respiratory failure; ATG, antithymocyte globulin; BAL, bronchoalveolar lavage; BUN, blood urea nitrogen; CBC, complete blood cell count; CMV, cytomegalovirus, D, donor; DM, diabetes mellitus; DVT, deep vein thrombosis; F, female; G-CSF, granulocyte-colony stimulating factor; Hb, hemoglobin; IVIG, intravenous immunoglobulin; M, male; max., maximum; MCV, mean corpuscular volume; MMF, mycophenolate mofetil; MSSA, methicillin-sensitive Staphylococcus aureus; Plts, platelets; R, recipient; RBC, red blood cell; RSV, respiratory syncytial virus; RV, right ventricular; s/p, status post; URI, upper respiratory tract infection; VATS, video-assisted thoracoscopic surgery; WBC, white blood cell count.