Hemophagocytic Syndrome in a Patient with ALL: Morphology Still Matters
We report a case of a young patient suffering from very-high risk B-acute lymphoblastic leukemia (ALL) refractory to first-line therapy with early central nervous system relapse, sequentially treated with inotuzumab. At this timepoint, the patient showed persistent transfusion-refractory thrombocyto...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-06-01
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| Series: | Hemato |
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| Online Access: | https://www.mdpi.com/2673-6357/2/2/20 |
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| author | Andrea Duminuco Giuseppe A. M. Palumbo Bruno Garibaldi Rosario Foti Francesco Di Raimondo Cinzia Maugeri Elisa Mauro Paolo F. Fiumara Calogero Vetro |
| author_facet | Andrea Duminuco Giuseppe A. M. Palumbo Bruno Garibaldi Rosario Foti Francesco Di Raimondo Cinzia Maugeri Elisa Mauro Paolo F. Fiumara Calogero Vetro |
| author_sort | Andrea Duminuco |
| collection | DOAJ |
| description | We report a case of a young patient suffering from very-high risk B-acute lymphoblastic leukemia (ALL) refractory to first-line therapy with early central nervous system relapse, sequentially treated with inotuzumab. At this timepoint, the patient showed persistent transfusion-refractory thrombocytopenia (platelets < 5000/uL), fever and cytomegalovirus infection. A bone marrow revaluation showed complete remission of ALL, but hemophagocytic elements and activated macrophages were present. At physical examination, the spleen was palpable. Blood chemistry showed hyperferritinemia (1419 ng/mL, normal range 24–336), NK cells suppression (11 cells/microL, minimum value 90) and IL-6 increase (119.1 pg/dL, normal values < 1.8). Triglycerides and fibrinogen were normal. A diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made according to HLH-2004 criteria. The patient was treated with the IL-1 inhibitor anakinra at escalating dosage. After an initial improvement in altered HLH parameters, a clinical worsening occurred with progressing thrombocytopenia and anemia and a remarkable increase in ferritin (4066 ng/mL). The patient then underwent rituximab-based salvage treatment but died due to HLH and B-ALL progression. HLH could be a rare complication in ALL patients. Despite increasing biological knowledge, prognosis remains poor, and more efforts are needed in order to improve survival in these patients. |
| first_indexed | 2024-03-10T10:48:47Z |
| format | Article |
| id | doaj.art-5d8ca7c1db22479ea5ca147760bc3dc5 |
| institution | Directory Open Access Journal |
| issn | 2673-6357 |
| language | English |
| last_indexed | 2024-03-10T10:48:47Z |
| publishDate | 2021-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Hemato |
| spelling | doaj.art-5d8ca7c1db22479ea5ca147760bc3dc52023-11-21T22:24:16ZengMDPI AGHemato2673-63572021-06-012234735210.3390/hemato2020020Hemophagocytic Syndrome in a Patient with ALL: Morphology Still MattersAndrea Duminuco0Giuseppe A. M. Palumbo1Bruno Garibaldi2Rosario Foti3Francesco Di Raimondo4Cinzia Maugeri5Elisa Mauro6Paolo F. Fiumara7Calogero Vetro8Post Graduation School of Hematology, University of Catania, A.O.U. “Policlinico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyDivision of Hematology, A.O.U. “Policlinico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyPost Graduation School of Hematology, University of Catania, A.O.U. “Policlinico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyDivision of Reumathology, A.O.U. “Policlinico-San Marco”, 95123 Catania, ItalyDivision of Hematology, A.O.U. “Policlinico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyDivision of Hematology, A.O.U. “Policlinico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyDivision of Hematology, A.O.U. “Policlinico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyDivision of Hematology, A.O.U. “Policlinico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyDivision of Hematology, A.O.U. “Policlinico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyWe report a case of a young patient suffering from very-high risk B-acute lymphoblastic leukemia (ALL) refractory to first-line therapy with early central nervous system relapse, sequentially treated with inotuzumab. At this timepoint, the patient showed persistent transfusion-refractory thrombocytopenia (platelets < 5000/uL), fever and cytomegalovirus infection. A bone marrow revaluation showed complete remission of ALL, but hemophagocytic elements and activated macrophages were present. At physical examination, the spleen was palpable. Blood chemistry showed hyperferritinemia (1419 ng/mL, normal range 24–336), NK cells suppression (11 cells/microL, minimum value 90) and IL-6 increase (119.1 pg/dL, normal values < 1.8). Triglycerides and fibrinogen were normal. A diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made according to HLH-2004 criteria. The patient was treated with the IL-1 inhibitor anakinra at escalating dosage. After an initial improvement in altered HLH parameters, a clinical worsening occurred with progressing thrombocytopenia and anemia and a remarkable increase in ferritin (4066 ng/mL). The patient then underwent rituximab-based salvage treatment but died due to HLH and B-ALL progression. HLH could be a rare complication in ALL patients. Despite increasing biological knowledge, prognosis remains poor, and more efforts are needed in order to improve survival in these patients.https://www.mdpi.com/2673-6357/2/2/20hemophagocytic lymphohistiocytosisacute lymphoblastic leukemiamorphologyIL-1 receptor antagonistanakinra |
| spellingShingle | Andrea Duminuco Giuseppe A. M. Palumbo Bruno Garibaldi Rosario Foti Francesco Di Raimondo Cinzia Maugeri Elisa Mauro Paolo F. Fiumara Calogero Vetro Hemophagocytic Syndrome in a Patient with ALL: Morphology Still Matters Hemato hemophagocytic lymphohistiocytosis acute lymphoblastic leukemia morphology IL-1 receptor antagonist anakinra |
| title | Hemophagocytic Syndrome in a Patient with ALL: Morphology Still Matters |
| title_full | Hemophagocytic Syndrome in a Patient with ALL: Morphology Still Matters |
| title_fullStr | Hemophagocytic Syndrome in a Patient with ALL: Morphology Still Matters |
| title_full_unstemmed | Hemophagocytic Syndrome in a Patient with ALL: Morphology Still Matters |
| title_short | Hemophagocytic Syndrome in a Patient with ALL: Morphology Still Matters |
| title_sort | hemophagocytic syndrome in a patient with all morphology still matters |
| topic | hemophagocytic lymphohistiocytosis acute lymphoblastic leukemia morphology IL-1 receptor antagonist anakinra |
| url | https://www.mdpi.com/2673-6357/2/2/20 |
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