About ALCL

Anaplastic large cell lymphoma (ALCL) is a sub-type of non-Hodgkin's Lymphoma. It's a cancer of the lymphatic system. ALCL is more common in children and young adults, but very rare in babies (average age of diagnosis is 9 years). Only five cases were reported in the past 10 years in babies in Europe. The chances of a baby getting ALCL are about 1 in 10 million. It develops when white blood cells called T-cell lymphocytes divide in an uncontrolled way. These build up in parts of the body like the lymph nodes, lungs or skin. The spread of the disease helps determine the stage of the disease. Quite often, ALCL is diagnosed at stage 3 or 4, which indicates considerable spread throughout the lymph nodes and organs. ALCL can be either ALK positive or ALK negative depending on whether the lymphoma cells have a protein called anaplastic lymphoma kinase (ALK) (Source: Macmillan cancer support). The ALK type and stage can help determine whether the patient is a high or low risk patient and, hence, the treatment. 

Lila has ALK positive ALCL. She is a high risk patient as her disease was classed as stage 4. Imaging of her body showed that the disease had spread beyond the pelvic lymph nodes to the thorax and neck and there was involvement of her kidneys, liver, spleen and lungs. Luckily there wasn't any CNS involvement (no white cells in the spinal fluid or her brain). We suspect her disease started around March when we detected a small skin lesion in her groin area. This was attributed by GPs and health visitors to nappy rash and was treated with antifungals and steroids. When these skin lesions are caught early in the disease they can be simply removed with surgery. If they spread they become systemic and they need chemotherapy. Unfortunately, Lila's ALCL is now systemic.

On the day of diagnosis Lila started chemotherapy. She had two cycles of COP (cyclophosphamide, vincristine, and prednisolone) with intrathecal treatment (treatment to the spinal fluid through a lumbar puncture) to help contain the cancer. This was followed by the ALCL 99 protocol for high risk patients. The ALCL 99 protocol is the standard chemotherapy for ALCL patients and has been found to be successful in numerous patients. The treatment works by targeting dividing cells (cancer cells as well as healthy dividing cells). More targeted treatments, which specifically target Lila's type of cancer, are now becoming available, but are still experimental in their nature as clinical trials are still ongoing. 

Lila's condition is also affected by a life threatening secondary disease called hemophagocytic lymphohistiocytosis (HLH), which often accompanies ALCL. With HLH, T and NK cells become overactive resulting in these cells damaging the own body's tissues and organs. In Lila, HLH manifests as an abnormally large spleen, very high ferritin and triglycerides and by the excessive consumption of red cells, which leads her to need blood transfusions very often. She's had 15 blood transfusions so far. The best way to treat the HLH is to treat the cancer. 

Before the start of the 3rd cycle her disease was found to be in partial remission (lesions in the thorax and liver had disappeared but there were still abnormal lymph nodes in the pelvic area and lesions in her kidneys). The spleen was still large. A decision was made to continue with the ALCL protocol to give the disease chance to remit further. However, before the start of the 4th cycle Lila started spiking temperatures again. Her ferritin increased to very high levels (around 8,000) and lymph nodes in her neck became swollen. An MRI showed that her disease was refractory (resistant) to the ALCL protocol and had spread again. 

Lila has now switched to a second line treatment, a combination of two drugs: 1. Vinblastine and 2. Crizotinib (an experimental drug that specifically targets Lila's type of cancer), and intrathecal treatment (which targets the central nervous system). Crizotinib is a targeted therapy (a monoclonal antibody) that inhibits the ALK protein. The drug was originally developed to treat ALK positive lung cancer (about 3% of lung cancer cases are caused by ALK). In some ways, we are fortunate that the ALK protein also happens to be a cause of lung cancer, as pharmaceutical companies don't generally develop drugs for childhood cancers. Initial results from clinical trials indicate that crizotinib can be an effective treatment strategy for ALCL. However, it is unknown whether crizotinib can cure ALCL. The current plan is to get Lila into remission in order to prepare her for a bone marrow transplant. It is thought that this is the only way to cure her. 

Lila started this process being very unwell. We did not think she would make it to a diagnosis. Luckily, the new second line treatment seems to have stabilised her. After a month she started smiling again and she slowly started catching up with baby milestones. However, as with every cancer patient, Lila has to deal with side effects of treatment on a daily basis. You can see below a picture of all the medications she takes daily. Furthermore, due to the effect of treatment on her immune system, she is also at high risk of infection. Infections are life threatening in cancer patients and require urgent medical attention.