How I treat microangiopathic hemolytic anemia in patients with cancer

Abstract

Microangiopathic hemolytic anemia (MAHA) with thrombocytopenia, suggests a thrombotic microangiopathy (TMA), linked with thrombus formation affecting small or larger vessels. In cancer patients, it may be directly related to the underlying malignancy (initial presentation or progressive disease), to its treatment, or a separate incidental diagnosis. It is vital to differentiate incidental thrombotic thrombocytopenia purpura or atypical hemolytic uremic syndrome in cancer patients presenting with a TMA, as they have different treatment strategies, and prompt initiation of treatment impacts outcome. In the oncology patient, widespread microvascular metastases or extensive bone marrow involvement can cause MAHA and thrombocytopenia. A disseminated intravascular coagulation (DIC) picture may be precipitated by sepsis or driven by the cancer itself. Cancer therapies may cause a TMA, either dose-dependent toxicity, or an idiosyncratic immune-mediated reaction due to drug-dependent antibodies. Many causes of TMA seen in the oncology patient do not respond to plasma exchange and, where feasible, treatment of the underlying malignancy is important in controlling both cancer-TMA or DIC driven disease. Drug-induced TMA should be considered and any putative causal agent stopped. We will discuss the differential diagnosis and treatment of MAHA in patients with cancer using clinical cases to highlight management principles.

Graphical abstract

Figure 1.

Summary of the laboratory features in patients with TMA. A differential diagnosis of the causes of a TMA are included, focusing specifically on cancer or its treatment and a summary of treatments beneficial in the individual subgroups. abdo, abdomen; adenoCa, adenocarcinoma; aPL, antiphospholipid; DAT, direct antiglobulin test; dsDNA, double-stranded DNA; ENA, extractable nuclear antigen; FBC, full blood count; GVHD, graft-versus-host disease; LMWH, low-molecular-weight heparin; PCR, polymerase chain reaction; RF, rheumatoid factor; SLE, systemic lupus erythematosus; STEC, Shiga toxin-producing Escherichia coli; U&E, urea and electrolytes; Vit, vitamin.