Pregnancy-associated hemophagocytic lymphohistiocytosis secondary to NK/T cells lymphoma

Abstract

Rationale:Hemophagocytic lymphohistiocytosis (HLH) occurs primarily in pediatric population, or secondary to malignancy, infection, or autoimmune disease. This disease is rare and prognosis is generally poor. Only a small number of cases during pregnancy have been reported in literature.Patient concerns:We report a case of pregnancy-associated HLH secondary to natural killer (NK)/T cells lymphoma. She was admitted at 30 weeks and 3 days of pregnancy with complaints of abdominal pain and fever as high as 39.2°C. The patient was found to have splenomegaly, pancytopenia, and acute hepatic failure.Diagnoses:A subsequent bone marrow biopsy revealed focal hemophagocytosis and atypical lymphoid cells. The splenic pulp also contained a large number of tissue cells proliferating and devouring mature red blood cells, lymphocytes, and cell debris. On the basis of these findings, we diagnosed the case as pregnancy-associated hemophagocytic lymphohistiocytosis secondary to NK/T cells lymphoma.Interventions:Treatment consisted with dexamethasone and etoposide in combination with rituximab.Outcomes:Due to timely termination of pregnancy, the neonate was in good condition. However, the patient died on the 18th day postoperation due to multiorgan failure.Lessons:We recommend that HLH be considered as differential diagnosis in a pregnant patient complaining of persistent fever, cytopenia, or declining clinical condition despite delivery of the baby. Prompt diagnosis and treatment is essential and fetal outcomes should also be considered. The decision to terminate a pregnancy and initiate chemotherapy during pregnancy with malignancy-associated HLH (M-HLH) needs to be further investigated in a larger cohort.

Etoposide combined with ruxolitinib for refractory hemophagocytic lymphohistiocytosis during pregnancy: a case report and literature review

“…As shown in Table 1, changes in blood count are common. All the 11 patients had abnormal hemogram, and there were 4 patients with Pancytopenia [8][9][10], 6 patients had anemia and thrombocytopenia [4,[11][12][13][14][15]. Only one patient had thrombocytopenia [10].…”

A Case of Recurrent Pregnancy-Induced Adult Onset Familial Hemophagocytic Lymphohistiocytosis

“…The diagnosis and onset of symptoms are typically manifest during the second trimester, usually resulting in spontaneous abortion, often with improvement in symptoms afterwards. The earliest reported case was at 10 weeks of gestation [ 10 - 12 , 15 - 18 ]. However, in our patient, she presented both times in her first trimester: first, she was diagnosed with HLH at a serum β-hCG level indicative of very early pregnancy (around 3 weeks); on the second she had an HLH flare when she had a confirmed pregnancy at 6 weeks of gestation.…”

“…The pathogenesis of pregnancy as a trigger for HLH is currently not well understood but multiple theories have been proposed [ 15 - 19 ]. One theory is that the immature placenta releases trophoblastic debris with fetal RNA and DNA components into maternal circulation, leading to a systemic inflammatory response [ 16 , 17 ].…”

Management of pregnancy-related hemophagocytic lymphohistiocytosis

“…(3) cytopenia (affecting ≥ 2 of 3 lineages in peripheral blood), with hemoglobin levels < 90 g/L, platelet count < 100 × 10 9 /L, and neutrophil count < 1.0 × 10 9 /L; (4) hypertriglyceridemia and/or hypofibrinogenemia, with fasting triglyceride levels ≥ 3.0 mmol/L and fibrinogen levels ≤ 1.5 g/L; (5) hemophagocytosis in the bone marrow, spleen, or lymph nodes, and no evidence of malignancy; (6) low or absent NK-cell activity (according to the local laboratory reference); 7 Differences between groups were assessed using the Student's t test. All statistical analyses were performed using SPSS (version 25.0, Chicago, IL, USA), with an alpha of 0.05 used as the cutoff for significance.…”

“…Pregnancy-related HLH is rare, and most of the relevant literature involves case reports [3][4][5][6]. HLH has symptoms similar to those of obstetric complications, including hemolysis, elevated liver enzymes, low platelet count (HELLP), and acute fatty liver.…”