Anamnesis: We present the case of a 75-year-old woman with a personal history of insulin-dependent type 2 diabetes mellitus, arterial hypertension, dyslipidaemia, ischaemic heart disease, appendectomy and cholecystectomy, who presented with fever of 1 month's evolution, accompanied in the last 6 months by constitutional syndrome (anorexia, asthenia and loss of about 21 kg of weight) and the appearance of multiple skin haematomas after minimal trauma.
Physical examination: Physical examination revealed intense mucocutaneous pallor, multiple skin haematomas, generalised diminished vesicular murmur with crackles in the right lung base on pulmonary auscultation and splenomegaly on palpation, without hepatomegaly or peripheral lymphadenopathy.
Complementary tests: Complementary tests showed the following results:
-CBC: a) red blood count: haemoglobin 7.2 g/dl, haematocrit 22%, red cells 2,710,000/µl, MCV 81.1 fl, MCH 26.6 pg, MCHC 32.8 g/dl, red cell distribution range 19.8%, reticulocytes 3.45% (107.000/µl); b) platelet series: platelets 11,000/µl; and c) white series: leukocytes 1,500/µl.
-Peripheral blood smear: a) red series: anisocytosis, 14% normoblasts, dacryocytes and some macroovalocytes; b) platelet series: giant platelets; c) white series: 52% segmented, 30% lymphocytes, 4% monocytes.
-ESR: 32 mm/hour.
-Biochemistry: glucose 67 mg/dl, urea 33 mg/dl, creatinine 0.4 mg/dl, urate 5.6 mg/dl, bilirubin 1.1 mg/dl, AST 141 IU/l, ALT 38 IU/l, alkaline phosphatase 245 IU/l, LDH 1650 IU/l, GGT 75 IU/l, total proteins 4.9 mg/dl, cholesterol 149 mg/dl, triglycerides 266 mg/dl, calcium 9.1 mg/dl, iron 62 mg/dl, tranferrin saturation index 34%, transferrin 143 mg/dl, ferritin 7597 µg/dl, vitamin B12 609 pg/ml, folate 1.81 ng/ml, alpha-fetoprotein < 1.
-Proteinogram: albumin 54.6%, alpha 1 globulin 3%, alpha 2 globulin 11.8%, beta globulin 15.4%, gamma globulin 15.2%.
-Chest X-ray: right basal pulmonary infiltrate.
-Abdominal ultrasound: multiple focal hepatic lesions with diffuse involvement of the liver parenchyma with normal liver size, marked splenomegaly, with no intra-abdominal lymphadenopathy.
Diagnosis: Given the presence of constitutional syndrome, fever, pancytopenia with evidence of bone marrow infiltration, myeloptysis (leukoerythroblastic reaction and dacryocytes in peripheral blood smears), and the presence of space-occupying lesions in the liver, it was decided to perform a bone marrow aspirate and biopsy.
However, before obtaining the definitive result of the bone marrow examination, the patient, despite haemotherapy support, evolved unfavourably and died 9 days after admission. An autopsy was not performed due to the express wishes of the family.
The bone marrow examination revealed the following data:
-Bone marrow aspirate: dry aspirate (suggestive of myelofibrosis).
-Bone marrow biopsy: hypercellular bone marrow parenchyma (5/5), with massive and diffuse infiltration of neoplastic cells of haematopoietic appearance, with myelofibrosis and necrosis phenomena. The tumour cells are large, with abundant eosinophilic cytoplasm and bulky, polymorphic nuclei, with prominent central nucleolus and abundant mitotic figures. No haemophagocytosis is evident.
Immunohistochemistry:
-CD45/ EMA/ LISOZYME (Golgi pattern)/ CD68: positive.
-CD79a/ CD10/ CD23/ CD43/ IgS/ CD15/ CD30/ CD45RO/ CD1a/ CD2/ CD3/ CD4/ CD8/ CD57/ CD 34/ MPX/ FACTOR VIII/ LMP1 (EBV)/ BCL2/ P53: negative.
-Cell proliferation rate (Ki 67) >80%.
Tumour cells are accompanied by reactive lymphoid T cells (CD45RO/ CD2/CD3/CD8/CD57 positive) and histiocytes (CD68 positive).
The definitive diagnosis on bone marrow examination was compatible with histiocytic sarcoma according to the World Health Organisation classification.
