Classic Hairy Cell Leukemia
Description
● General Details
• Illness: Hairy Cell Leukaemia (HCL), an uncommon, slow-growing B-cell tumour.
• Incidence: 600 to 800 new cases each year in the United States.
• Epidemiology: Men in their middle years (M:F = 4:1), median age ~55.
• Pathology: Infiltration of bone marrow, spleen, and blood by neoplastic B-cells with "hairy" cytoplasmic projections.
• Variants: Classic HCL (HCLc), which often has an indolent course and a BRAF V600E mutation.
The HCL variation (HCLv) is more aggressive, has a worse response to treatment, and does not have a BRAF mutation.
● Patient Case
• Age/Sex: man, 80 years old.
• History: Nine years prior, HCL was diagnosed.
• Cladribine treatment resulted in a partial response.
• Pentostatin + rituximab after relapse.
• Later relapse → rituximab + cladribine, accompanied by cerebral haemorrhage and neutropenia.
• Comorbidities include sleep apnoea, diabetes, and high blood pressure.
• Presentation: headache, impaired vision, fatigue, dizziness, and SOB.
● Examination & Investigations
➣Results:
•22 cm of splenomegaly, palpable above the umbilicus.
•bruises, lymphadenopathy, and petechiae.
•Leukocytosis severe (WBC 371,700 × 10⁹/L).
•thrombocytopenia (Plt 99,000 × 10⁹/L) and anaemia (Hb 6.4 g/dl).
➣blood smear:The "hairy" lymphocytes
➣Imaging:
•CT chest showing pleural effusion and bilateral ground-glass opacities.
•Multiple soft tissue masses (cerebellum, frontal, and temporal lobes) are seen on a CT/MRI scan of the brain.
•Monoclonal B-cell population (CD11c, CD103, aberrant CD5, incomplete CD10; CD25 missing) as determined by flow cytometry.
➣Mutation: positive for BRAF V600E.
● Discussion & Learning Points
➣First-line treatment:
• Purine analogues (pentostatin, cladribine) → long-lasting remissions.
• Relapse: More toxicity and less effectiveness.
• Although extremely uncommon, CNS involvement in HCL often denotes a severe illness.
➣Treatment options:
• Purine analogues + Rituximab.
•Ibrutinib (sometimes used, but may cause bleeding).
•There is little information about doxetumomab's (anti-CD22) CNS involvement.
• Vemurafenib, a BRAF inhibitor, is effective even at lower dosages and has been demonstrated to pass the blood-brain barrier.
• Other treatments mentioned in the literature include steroids, whole brain radiation, interferon-α, and intrathecal methotrexate.
• Vemurafenib side effects include arthralgia, nausea, rash or photosensitivity, baldness, and exhaustion.
➣ Duration of therapy:
• ~16–18 weeks, median response in 8–12 weeks.
• Case summary: Low-dose vemurafenib can safely and successfully elicit remission in elderly patients with CNS lesions, even if they are weak.
Protocol
● Management
➣Acute Leukostasis:
• 2 g/day of hydroxyurea.
• After three sessions of leukapheresis, the WBC dropped to 89,000 × 10⁹/L, and the symptoms improved.
➣CNS Involvement:
•Ibrutinib (stopped due to bleeding complications).
• Vemurafenib (BRAF inhibitor):
• well tolerated; started at 240 mg BID.
• Increased to 480 mg BID → notable progress.
➣Response:
• Neurologic symptoms resolved in 1 month.
• Three-month MRI: All CNS lesions have disappeared.
• Spleen size decreased, Hb returned to normal, and WBC fell to 28,400 × 10⁹/L.
• Retinopathy has been resolved.
● Problems Faced
•severe exhaustion, headache, dizziness, shortness of breath, and blurred vision as a result of a CNS involvement and HCL recurrence.
•severe thrombocytopenia, anaemia, and leukocytosis that compromise leukostasis and raise the risk of bleeding.
•severe splenomegaly that is producing pain in the abdomen.
•Breathlessness brought on by leukostasis is getting worse.
•complications from previous cerebral haemorrhage and neutropenic fever.
•Given the significant disease load, there is a risk of tumour lysis syndrome.
•haemorrhage and thrombocytopenia while using ibrutinib.
•medical weakness and biopsy procedure danger.
•Uncertainty in diagnosis: separating malignant from infectious CNS lesions.
•limited alternatives for therapy because of the age, comorbidities, and adverse drug reactions of the patient.
● Nursing Management
➣Issue: Extreme weakness and exhaustion (caused by anaemia and the load of sickness)
➣Interventions:
•Use a standardised scale, such as the FACIT-F, to measure your degree of fatigue every day.
•Plan rest moments in between activities to promote energy saving.
•When the patient is too weak, help them with ADLs like dressing and bathing.
•Encourage a diet rich in calories and protein to boost energy levels.
•For Hb < 7–8 g/dL, give blood transfusions as directed.
•Teach the patient's relatives how to help with physical duties.
● Issue: Infection Risk (immunosuppressive medication, neutropenia)
➣ Interventions:
• Keep an eye on your temperature every day, your CBC (ANC levels), and any early symptoms of an infection (dysuria, cough, sore throat).
• Keep your hands clean for both patients and guests.
• Use protective isolation if the ANC is less than 500/mm³.
Serve a neutropenic diet (avoid undercooked meat and raw fruits and vegetables).
• Teach the patient to stay away from crowded places, ill people, and dirty food.
• As directed by protocol, give preventative antibiotics and antivirals.
• Promote prompt immunisation.
● Issue: Bleeding Risk (reticular haemorrhage, petechiae, bruises, thrombocytopenia)
➣ Interventions:
• Check every day for petechiae, haematuria, melena, and bleeding from the gums and nostrils.
• If your platelets are less than 50,000, stay away from invasive operations, rectal temperatures, and intramuscular injections.
• Avoid hard meals, use an electric razor, and provide a soft toothbrush.
• When there is active bleeding or a platelet count below 20,000, have an emergency platelet transfusion on hand.
• Inform the patient and their family on how to prevent falls and how to stop bleeding.
● Issue: Respiratory distress and dyspnoea (caused by pleural effusions, splenomegaly, and leukostasis)
➣ Interventions:
• Regularly check lung sounds, SpO₂, and breathing rate.
• To facilitate breathing, place the patient in semi-Fowler's position.
• As directed, administer oxygen treatment.
• As directed, prepare for and help with leukapheresis.
• Follow the guidelines while administering hydroxyurea for cytoreduction.
• During dyspnoea, teach breathing and relaxation strategies to help patients feel less anxious.
● Issue: Neurological Symptoms (headache, lightheadedness, altered eyesight from central nervous system involvement)
➣ Interventions:
• Conduct neurological evaluations (cranial nerve, GCS, and motor/sensory state) throughout each shift.
• Keep an eye out for symptoms of elevated intracranial pressure (ICP), such as nausea, disorientation, or impaired vision.
• If necessary, get ready for a lumbar puncture or biopsy and help with CT/MRI procedures.
• Deliver recommended drugs (steroids, vemurafenib) and keep an eye out for any effects.
• Teach the patient and their family to report any new neurological problems right away.
● Issue: Abdominal Pain and Splenomegaly
➣ Interventions:
• Regularly check the size of the spleen and measure the circumference of the abdomen.
• Teach the patient to prevent abdominal trauma, which increases the chance of splenic rupture.
• In order to control pain, provide analgesics.
• To lessen the discomfort caused by fullness in the abdomen, promote modest, frequent meals.
• Get the patient ready for CT/US imaging to check the size of the spleen.
● Issue: Nutritional Deficit (eating less, splenomegaly causing early satiety, adverse effects of therapy)
➣ Interventions:
• Evaluate weight, BMI, and food consumption every week.
• Promote frequent, short, nutrient-dense meals.
• Work together with a dietitian to create a diet rich in iron and protein.
• Offer dietary supplements, such as fortified beverages and oral protein smoothies.
• If chemotherapy is causing nausea, use antiemetics before meals.
● Issue: Psychological Distress (prognostic anxiety, relapses, and chronic disease)
➣ Interventions:
• Offer active listening, emotional support, and referrals to counselling.
• Encourage candid conversations and include family members in care planning.
• Instruct coping mechanisms such as journaling, relaxation, and meditation.
• Consult with leukaemia support groups.
• Deal sympathetically with issues related to body image, such as baldness, bruises, and weight fluctuations.
● Issue: Side effects of medication (rash, photosensitivity, arthralgia, baldness, vemurafenib)
➣ Interventions:
• Advise the patient to wear protective clothes and sunscreen, and keep an eye out for skin rashes and photosensitivity responses.
• Offer gentle shampoo for alopecia and moisturisers for dry skin.
• Use prescription painkillers and mild workouts to treat joint discomfort.
• Teach the patient to take their medications as prescribed and to report any adverse effects as soon as possible.
● Issue: Lack of knowledge about the illness process, treatment, and self-care
➣ Interventions:
• Provide information about BRAF mutation, HCL, treatment strategy, and follow-up.
• Provide instruction on how to prevent infections, how to stop bleeding, and how to change your diet.
• Give senior patients written instructions and reiterate important details.
• Encourage the patient and their family to keep a journal of their symptoms.
• Stress the value of bone marrow biopsies and follow-up appointments for confirming remission.
Notes
For more details visit 10.3389/fonc.2022.1100577
