2017, Number 3
Hemophagocytic lymphohistiocytosis in a patient with myelomatous activity after autologous bone marrow transplant
Motolinia-Muñoz Y, Gastélum-Cano JM, Tenorio-Páez C, Ruiz-Argüelles GJ
Language: English
References: 14
Page: 139-145
PDF size: 360.88 Kb.
ABSTRACT
Hemophagocytic lymphohistiocytosis is a difficult diagnostic and infrequent hyperinflammatory syndrome. Patients with malignant hematological diseases are especially prone to develop it. Multiple myeloma (MM) is reported among diseases that present association with secondary HLH (sHLH). However, this association is poorly studied. Here we describe the case of a 68-year-old male patient, Mexican mestizo, who was diagnosed with MM in 2008 and attended to the Center for Hematology and Internal Medicine for evaluation. The patient reported weight loss of 9 kg, fatigue and weakness. Laboratory studies showed serum protein electrophoresis (sPEP) without monoclonal band pattern; urine immunofixation electrophoresis test (uIFE) positive for gamma heavy chain and kappa light chain; serum immunofixation electrophoresis test (sIFE) negative. Bone marrow biopsy and aspiration showed a normocellular bone marrow with 1.2% of plasmatic cells. Bone marrow immunophenotyping by flow cytometry found no neoplasic cells. The X-ray scanning showed a radiolucid area in cranium, probably corresponding to osteolytic lesions; spine with crushed lumbar discs and ilium osteolysis. In May 18th of 2016 new findings in laboratories studies including sIFE positive for IgG-Lambda and sPEP with monoclonal band pattern in gamma fraction were reported. An outpatient autologous peripheral blood stem cell transplantation (APBSCT) was performed in February 11th of 2017. On Day + 4 he was admitted at intensive care unit for transplant subsequent events. A new bone marrow biopsy and aspiration was carried out and showed 32.3% of plasmatic cells. Histiocytes with Hemophagocytic phenomenon were observed. To the knowledge of the authors, this case is de sixth associated to myelomatous activity and the third after APBSCT.REFERENCES