Hemophagocytic lymphohistiocytosis and myelodysplastic syndrome: a case report and review of the literature

Abstract

Background Hemophagocytic lymphohistiocytosis (HLH) is characterized by hyperinflammation and life-threatening cytopenias. Survival is poor, and management is pivotal on rapid identification of the disease. HLH is associated with hematologic malignancies, however correlation with myelodysplastic syndromes (MDS) is exceedingly unusual. Although minimizing overwhelming hyperinflammation by treating hemophagocytosis are central for HLH outcome, there is urgent necessity to identify potential initiating mechanisms that could assist in therapy design. Case description Here, we describe an elderly African American patient who developed rapid onset of cytopenias and coagulopathy associated with hepatic and bone marrow hemophagocytosis. We analyze four additional similar cases to isolate clinical, laboratory and cytogenetic findings expected in patients exhibiting concurrent HLH and MDS. HLH linked with MDS retains common HLH features associated with systemic hyperinflammation such as fever, hypotension, hepatosplenomegaly, hyperferritinemia, coagulopathy and rapidly evolving cytopenias. Typical MDS chromosomic abnormality such as trisomy 8 was frequently observed in our studied cases. Conclusion Our case describes difficulties while managing HLH in MDS patients. Diagnosis should be based on identifying HLH appropriate criteria and if possible karyotypic abnormalities normally observed in MDS.

Hemophagocytic Lymphohistiocytosis in A Post Covid-19 Patient with Mds: A Case Report

“…As for the remaining 2 cases, they were considered as intermediate and low risk MDS according to R-IPSS, with no further information given regarding WHO classification. Furthermore, in 75% of MDS-related sHLH published cases organomegaly was profound in clinical examination [7,[16][17][18].…”

“…Bone marrow smears revealed hemophagocytosis (Figure 1). Bone marrow karyotype was normal (46,XX [20]) [7]. Trephine biopsy showed signs of erythroid dysplasia along with the presence of granulomas.…”

“…Many factors have been implicated in the development of the syndrome, including infections by fungi, protozoa, bacteria and viruses, autoimmune diseases and hematological or non-hematological malignancies [5]. At the same time, reports of myelodysplastic syndrome as a causal factor are rare [7].…”

A Case of Secondary Hemophagocytic Lymphohistiocytosis Presenting with Severe Dyserythropoeisis in a Patient with Autoimmune Hemolysis

“…This is an important point as obvious dysplasia is easily identified and may serve as a marker of profound bone marrow stress providing evidence supporting the diagnosis and prompting a more thorough review when obvious hemophagocytic cells are not initially identified. It is also worth noting that other groups have identified myeloid neoplasms which may also present with dyserythropoeisis such as MDS, AML, and MPNs as triggers for HLH [ 9 , 10 ] making their exclusion an important part of the diagnostic workup.…”

Myelodysplastic syndrome associated‐haemophagocytic lymphohistiocytosis: A retrospective study of 15 cases in a single centre

“…4,5 HLH initiated by MDS (MDS-HLH), as a subtype of M-HLH, 6 is a relatively rare occurrence and has been underexplored. To our knowledge, the previous literature consisted of only isolated case reports, 7 resulting in limited understanding of its underlying pathogenesis and optimal treatment strategies. Prompted by a cohort of patients, our research represents the first clinical study exclusively dedicated to MDS-HLH.…”