| 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 |
Case Report
Volume 4, Number 1, March 2015, pages 151-154
Hemophagocytic Lymphohistiocytosis Secondary to Diffuse B-Cell Lymphoma
Tables
| The diagnosis of HLH can be established if one of either 1 or 2 is fulfilled. |
| 1. A molecular diagnosis consistent with HLH is made. |
| 2. Five out of the eight criteria (below) are fulfilled. |
| i. Fever |
| ii. Splenomegaly |
| iii. Cytopenias affecting 2 - 3 lineages in peripheral blood |
| Hemoglobin < 9 g/L |
| Platelets < 100 × 109/L |
| Neutrophils < 1.0 × 109/L |
| iv. Hypertriglyceridemia and/or hypofibrinogenemia: fasting triglycerides ≥ 3.0 mmol/L (265 mg/dL), fibrinogen ≤ 1.5 g/L |
| v. Hemophagocytosis in bone marrow, spleen or lymph nodes |
| vi. Low or absent natural killer (NK) cell activity |
| vii. Ferritin ≥ 500 μg/L |
| viii. Soluble CD25 (soluble IL 2 receptor) > 2,400 U/mL |
| 1. High fever for more than a week (peak > 38.5 °C) |
| 2. Anemia, hemoglobin < 9 g/dL or thrombocytopenia, platelets < 100,000/μL |
| 3. |
| i. Lactate dehydrogenase (LDH) > 2 × upper limit |
| ii. Ferritin > 1,000 ng/dL |
| iii. Hepatosplenomegaly on imaging |
| iv. Fibrin degradation product (FDP) > 10 μg/mL |
| 4. Hemophagocytosis in bone marrow, liver or spleen |
| 5. No evidence of infection |
| 6. Histopathologically confirmed malignant lymphoma |
| A diagnosis of LAHS requires that all of the above items are fulfilled. Of item 3, at least two of the four sub-items (i-iv) should be fulfilled. When items 1 to 5 are present for 2 weeks and glucocorticoids and gamma globulin therapy is not effective, a diagnosis of probable LAHS can be made and chemotherapy against malignant lymphoma can be started. |