| Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access |
| Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc |
| Journal website http://www.thejh.org |
Original Article
Volume 8, Number 4, December 2019, pages 149-154
Hemophagocytic Lymphohistiocytosis in Adults: Associated Diagnoses and Outcomes, a Ten-Year Experience at a Single Institution
Tables
| Criteria | No. (%) of patientsa |
|---|---|
| aTotals less than 41 indicated that not all patients were assessed/tested for that specific criterion. | |
| Fever (temperature ≥ 38.5°C) | 32/41 (78%) |
| Splenomegaly | 23/40 (56%) |
| Cytopenias, of at least two cell lines: | 29/41 (71%) |
| HgB < 9 g/dL | 39/41 (95%) |
| Platelet count < 100,000/µL | 31/41 (76%) |
| Absolute neutrophil count < 1,000/µL | 9/41 (22%) |
| Hypertriglyceridemia (> 265 mg/dL) and/or hypofibrinogenemia (≤ 150 mg/dL) | 26/41 (63%) |
| Hemophagocytosis in bone marrow, spleen, lymph node, or liver | 29/41 (71%) |
| Low or absent NK cell activity (< 10 lytic units) | 15/18 (83%) |
| Ferritin ≥ 500 µg/L | 41/41 (100%) |
| Elevated soluble CD25 (soluble interleukin-2 receptor) ≥ 2,400 U/mL | 15/24 (62%) |
| Hepatomegaly (clinically palpable liver or seen on imaging) | 23/41 (56%) |
| Monocytosis (absolute monocyte > 1,000/µL) | 8/40 (20%) |
| Renal failure (50% increase in creatinine over baseline) | 21/41 (51%) |
| Elevated hepatic enzymes (≥ 2.5 times the upper limit of normal) | 25/41 (61%) |
| Coagulopathy (prothrombin time ≥ 1.5 times upper limit of normal and/or partial thromboplastin time ≥ 1.5 times upper limit of normal, and/or D-dimer ≥ 10.0 µg/mL) | 11/41 (27%) |
| Hypoalbuminemia (< 3.5 g/dL) | 40/40 (100%) |
| Elevated lactate dehydrogenase (LDH) (≥ 2.5 times upper limit of normal) | 25/36 (69%) |
| Elevated β2 microglobulin (≥ 2 mg/L) | No patient was tested |
| Condition | Number of patients |
|---|---|
| Malignancy (n = 16) | |
| Lymphoma (n = 11) | |
| Peripheral T-cell lymphoma | 3 |
| Diffuse large B-cell lymphoma | 2 |
| Hodgkin lymphoma | 2 |
| Post-transplant lymphoproliferative disorder | 2 |
| Mantle cell lymphoma | 1 |
| T/natural killer cell lymphoma | 1 |
| Leukemia (n = 3) | |
| Acute lymphoblastic leukemia | 1 |
| Acute myeloid leukemia | 1 |
| Chronic lymphocytic leukemia | 1 |
| Solid organ cancer (n = 2) | |
| Renal cell carcinoma | 1 |
| Ovarian cancer | 1 |
| Infection (n = 22) | |
| Viral (n = 17) | |
| Epstein-Barr virus | 8 |
| Cytomegalovirus | 5 |
| Hepatitis B | 1 |
| Human herpes virus 6 | 1 |
| Influenza | 1 |
| Parvovirus | 1 |
| Bacterial (n = 4) | |
| Vancomycin-resistant enterococci | 2 |
| Methicillin-resistant Staphylococcus aureus | 1 |
| Ehrlichiosis | 1 |
| Fungal (n = 1) | |
| Histoplasmosis | 1 |
| Autoimmune disorder (n = 8) | |
| Stills disease | 4 |
| Systemic lupus erythematosus | 2 |
| Myasthenia gravis | 1 |
| Sweet syndrome | 1 |
| History of transplant (n = 9) | |
| Liver | 3 |
| Bone marrow | 2 |
| Lung | 2 |
| Kidney | 1 |
| Heart | 1 |
| Aplastic anemia | 1 |
| Type of treatment | No. of patients |
|---|---|
| Corticosteroids alone | 15 |
| Corticosteroids along with chemotherapy (n = 18) | |
| Dexamethasone and etoposide | 6 |
| Cyclophosphamide, doxorubicin, vincristine and prednisone | 2 |
| Rituximab, etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin and intrathecal methotrexate | 2 |
| Dexamethasone, vincristine and dasatinib | 1 |
| Methylprednisolone, rituximab, etoposide and intravenous immunoglobulin | 1 |
| Methylprednisolone, methotrexate and tocilizumab | 1 |
| Methylprednisolone and rituximab | 1 |
| Methylprednisolone, tumor necrosis factor alpha inhibitor, cyclosporine, anakinra, tocilizumab | 1 |
| Methylprednisolone and cyclosporine | 1 |
| Cyclophosphamide, doxorubicin, vincristine, etoposide and prednisone | 1 |
| Dexamethasone, rituximab, doxorubicin, vinblastine, dacarbazine | 1 |
| Cisplatin, etoposide, gemcitabine, methylprednisolone | 1 |
| Anakinra and prednisone | 1 |
| Etoposide, prednisolone, vincristine, cyclophosphamide and doxorubicin for one cycle, then brentuximab | 1 |
| Corticosteroids along with antiviral | 3 |
| Cytotoxic chemotherapy alone | 2 |
| Azacitidine | 1 |
| Cladribine and cytarabine | 1 |
| Intravenous immunoglobulin alone | 1 |
| No treatment | 2 |
| Median survival, all patients (days) | 1,095 |
| Survived, initial hemophagocytic lymphohistiocytosis hospitalization | 25 (61%) |
| Remission | 21/25 (84%) |
| Died (due to any cause), following hospital discharge | 7/25 (28%) |
| Median survival of those who died following hospital discharge (days) | 240 |
| Died, initial hemophagocytic lymphohistiocytosis hospitalization | 16 (39%) |
| Sepsis or multi-organ failure | 16/16 (100%) |
| Pneumonia and/or respiratory failure | 7/16 (43.7%) |
| Malignancy-associated | 6/16 (37.5%) |
| Medication-induced | 1/16 (6%) Stevens-Johnson syndrome vs. toxic epidermal necrolysis |