|Year : 2019 | Volume
| Issue : 2 | Page : 180-181
Severe hyperlipidemia in a case of acute lymphoblastic leukemia
Rimjhim Sonowal, Vineeta Gupta
Division of Hematology Oncology, Department of Pediatrics, Institute of Medical Sciences, BHU, Varanasi, Uttar Pradesh, India
|Date of Web Publication||2-May-2019|
Division of Hematology Oncology, Department of Pediatrics, Institute of Medical Sciences, BHU, Varanasi, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Severe hyperlipidemia (>1000 mg/dL) at initial presentation of acute lymphoblastic leukemia (ALL) is rare. Cases of hyperlipidemia during therapy for childhood ALL where they were secondary to L-asparaginase or steroids have been described. This is a case report of a one-and-half-year-old boy who presented to us with fever, abdominal distension, severe pallor, and hepatosplenomegaly. Although his investigations were suggestive of ALL, the initial blood samples were found to be grossly lipemic. The lipid profile was abnormal, showing severe hypertriglyceridemia (serum triglycerides 1552 mg/dL). High-density lipoprotein and low-density lipoprotein levels were low, but there were raised very low-density lipoprotein level and serum lactate dehydrogenase (18117 U/L). The patient was started on induction of remission with careful monitoring of biochemical parameters. Abnormal lipid levels declined gradually with normalization of the levels at the end of one week of chemotherapy. No further complications were encountered during the course of induction of remission.
Keywords: Acute lymphoblastic leukemia, high-density lipoprotein, hypertriglyceridemia, very low-density lipoprotein
|How to cite this article:|
Sonowal R, Gupta V. Severe hyperlipidemia in a case of acute lymphoblastic leukemia. Indian J Cancer 2019;56:180-1
| » Introduction|| |
Abnormal lipid levels in blood samples have been associated with various forms of cancer,,, the mechanism for such perturbations of lipid metabolism and their clinical significance are not known. However, the altered lipid levels observed in blood at diagnosis led to the speculation that these may serve as biochemical markers of acute lymphoblastic leukemia (ALL), may aid in the assessment of minimal residual disease and the prediction of recurrence, and may correlate inversely with the effectiveness of chemotherapy. Asparaginase and steroids can also cause hypertriglyceridaemia in children with ALL. However, hypertriglyceridemia at initial diagnosis of ALL is very rare. We report a case of ALL in a child who had severe hypertriglyceridemia at the time of initial diagnosis.
| » Case Report|| |
A one-and-half-year-old boy presented with fever and abdominal distension since 1 month. He was apparently well 1 month back before he developed moderate to high-grade fever with insidious onset but not associated with chills and rigors, and responded to medication. He also had abdominal swelling which was gradual in onset and progressive in nature. On examination, he was a normal built child with severe pallor and mild pedal edema. On systemic examination, he had hepatosplenomegaly (liver 11 cm, spleen 5 cm) and other systems were within normal limits. On the basis of history and examination, a possibility of ALL was kept in mind and the child was investigated accordingly. On the first day of admission, while drawing blood for investigations we found that the sample was grossly lipemic. A lipid profile test was conducted along with other tests. The results showed that total cholesterol level was 222 mg/dl (normal value<170 mg/dl in children), triglycerides was 1552 mg/dl (reference range 10-200), HDL- 18mg/dl (35-65), LDL- 53mg/dl (60-160), VLDL- 310 (5-45), LDH- 18117 U/L, uric acid- 10.5 mg/dl (2.5-7.5). Liver function tests were normal except for mild elevation of enzymes (SGOT, SGPT and Alkaline phosphatase). Renal function tests were almost normal with only mild elevation of blood urea. Serum ferritin level was 220 ng/ml (referrence range 24-336ng/ml in man and 11-307 ng/ml in women). Serum fibrinogen level was not evaluated. Initial total leucocyte count was 59,310/mm3 with platelet count of 36,000/mm3. Peripheral smear and bone marrow aspiration were suggestive of ALL with more than 80% lymphoblasts. There was no evidence of hemophagocytes in the bone marrow aspirate. Flow cytometry analysis showed expression of CD10 92%, CD19 97%, CD20 24% with features suggestive of B-ALL. Multiplex polymerase chain reaction analysis showed t(1;19) (q23;p13.3); TCF3-PBX1(E2A-PBX1) was positive. Cerebrospinal fluid cytospin for blasts was negative.
In view of high counts, the child was started on high-risk protocol with careful monitoring of biochemical parameters. Triglyceride levels decreased to 588 mg/dL and VLDL to 118 mg/dL on the third day of chemotherapy. At the end of first week of chemotherapy, lipid levels normalized. No further complications were observed during induction of remission.
| » Discussion|| |
Extreme hyperlipidemia with lipemic sample in association with leukemia at initial diagnosis is rare in children. We are aware of only single case report where a case of one-and-half-year-boy with advanced ALL who had lipemic sample was reported. In this boy, fatty acid analysis of the triglyceride esters revealed a high proportion of steric acid, which was associated with a slower in vitro degradation of VLDL by human milk lipoprotein lipase. This suggested that a cause of hyperlipidemia could be abnormal composition of triglycerides which render the VLDL a poor substrate for lipoprotein lipase. In our patient of ALL, hyperlipidemia was mainly due to hypertriglyceridemia with raised VLDL and low HDL levels. Hypertriglyceridemia is one of the diagnostic criteria of hemophagocytic lymphohistiocytosis (HLH) associated with certain malignancies. Currently, there are no generally accepted criteria for the definition of malignancy-triggered HLH or HLH during chemotherapy. The HLH-2004 criteria may serve as a substitute definition but they have substantial weaknesses. In the context of malignancy, several of these characteristics may be present and caused by the HLH and/or by the neoplasm. For clinical purposes, it is thus crucial to judge whether the combination, the extent, and the progression of the clinical and laboratory abnormalities are unusual, unexpected, and otherwise unexplained. In children, T-cell malignancies predominate the causes of malignancy associated HLH., Our patient presented with leukocytosis. His serum ferritin levels were not at par with diagnosis of HLH. Bone marrow aspirate showed no hemophagocytes. Flow cytometry analysis was suggestive of B-ALL. Hyperlipidemia showed rapid response to chemotherapy. Halton et al. demonstrated an altered blood lipid profile in children with ALL at diagnosis. A prospective study carried out in children with ALL showed lipid abnormalities which returned to normal on completion of chemo therapy. The study concluded that abnormal lipid levels may reflect nutritional status or altered lipid metabolism. Another study by Favrot et al. reported increase of triglycerides and VLDL at the time of diagnosis in children with ALL but found no such alterations in children with solid tumors. Investigations in adult patients with ALL have also shown an association between the reversal of altered lipid profiles and remission status. After the induction treatment, Baroni S, et al. found a significant increase of HDL-cholesterol (HDL-C) and apolipoprotein A1 (Apo A1) values only in those patients that achieved a complete remission. These results support the idea that some serum lipids, such as HDL-C and Apo A1, may have a role as early and reliable markers of the effectiveness of chemotherapy. Spiegel et al. found that the degree of lipid abnormality in ALL and non-Hodgkin's lymphoma was directly related to the underlying tumor burden and particularly to the presence of bone marrow involvement. However, even patients with minimal tumor bulk demonstrated plasma lipid abnormalities. The results suggest that an abnormality in systemic lipid metabolism, possibly in triglyceride clearance, is present in these patients and that its incidence in this population is high. Some studies have shown that treatment with l-asparaginase is associated with severe hyperlipidemia.,, One of the studies reported mild hypercholesteremia and low HDL levels after treatment for ALL. Hyperlipemia during the induction phase of treatment was found to be transient. A higher incidence of thrombotic complications was thought to be associated with hyperlipidemia secondary to asparaginase therapy. However, in our patient no such complication was observed, in spite of severe hyperlipidemia, and the induction was uneventful.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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