Physicians Awareness To Haematology (PATH) Newsletter
Dear friends and seniors,
Hope you all are doing great and taking care of yourselves and your family in this pandemic. We all are adapting to the changing practices in the current scenario. It gives me great pleasure in introducing this unique newsletter which will highlight the recent advances in the field of haemato-oncology and also we will discuss interesting day to day cases which will help us all to understand this subject better. I take this opportunity to thank each one of you for your continued support to our Jeevan Amrut Haematology Centre, which celebrated its Third year of existence recently.
Aims and objectives:
- To create awareness about Haematology and Oncology.
- Continuing Medical Education (CME).
- To update the medical community about latest developments in diagnostic haematology.
In this issue I will present two interesting cases of increase in blood counts – Erythrocytosis and Leucocytosis
43 yr old male, resident of Phulambri, police officer by occupation was referred by Urologist for persistent leucocytosis. The story started 2 months ago when he first had low grade fever and mild leucocytosis with a WBC count of 18,200. He is a smoker (stopped few years ago). No Diabetes Mellitus (DM) and was on one antihypertensive since 2 years. Oral antibiotics were given but no response so got admitted under physicians care. He complained of weakness and fatigue. No history of blood loss. He was started on IV antibiotics and had complete fever workup which showed nothing except found to have a renal calculus suspected to be the source of infection so referred to urology. He was afebrile but as counts were progressively increasing – started on meropenam and planned for surgery for renal stone. For pre-anesthesia fittness haematologist was involved.
On examination: He looked stable with normal vital parameters . No splenomegaly. No other abnormality. USG was normal.
CBC was – Hb 14.2, TLC 40,300, Platelet count – 5.11 Lakh, PBS showed leucocytosis with shift to left and 1 % blasts.
Evaluation – Planned for Bone marrow evaluation suspecting chronic myleloproliferative disease – CML –CP as peripheral smear showed shift to left with premature cells with increase in eosinophils and basophils. BM aspirate and biopsy both were hypercellular, FISH for BCR/ABL – for Philadelphia chromosome was positive – confirming the diagnosis of Chronic Myeloid Leukemia in Chronic phase
Treatment: Oral treatment with Imatinib 400 mg in tablet form once a day was started. One month later his CBC was completing normal.
Take home message:
- Leucocytosis is not always due to infection.
- Malignancy can cause low grade fever and leukocytosis.
- We should think of alternative diagnosis especially if not responding to conventional line of management
- Looking in to the peripheral smear always gives some clue
- High degree of suspicion is needed for early diagnosis of Malignancy.
Case 2 :
46 yr old male was referred for high hemoglobin (polycythemia) to our OPD. He was seen by a dermatologist 7 days back with chronic long standing pruritus and on investigations he had Hb of 19.4. His WBC was 15,100 and platelet count of 5.2 lakhs. He was a non smoker and did not consume alcohol. He did not have family history of thrombosis. He did not have palpable splenomegaly and no renal disease. He had these symptoms sincemany years with consultation from many consultants from various pathies. Itching use to be more in winter and after morning shower.
Investigations: His USG showed mild splenomegaly. His uric acid was 6.9. LFT and Creatinine were normal. Serum Erythropoietin was very low (this is a hormone produced by our kidney to control the Red blood cell production ) and a special test called JAK 2 mutation study was positive (JAK2 is a gene which regulates the red cell growth in bone marrow and if it is mutated can lead to “unregulated” red blood cell production and hence polycythemia). He was diagnosed to have “Primary Polycythemia” leading to aqua genic pruritus
Treatment: He underwent venesection or phlebotomy (to remove blood as we do in blood bank, but this blood which is removed cannot be used !!). He was started on hydroxyurea therapy to reduce the red blood cell production and the aim is to maintain the Hematocrit at <50 % level. He will need lifelong hydroxyurea therapy. He will also need Aspirin therapy lifelong. A new drug JAK2 inhibitor called “Jakavi” is now available in India for such diseases but not needed in his case. He was symptomatically so relieved with Hydroxyurea.
Many patients with polycythemia have high wbc and platelets also. Some of these patients with myeloproliferative disorders can progress to acute leukemia or myelofibrosis and hence need to be monitored closely.
The underlying cause of aquagenic pruritus is unknown. In some cases, it is a symptom of polycythemia vera or another underlying condition. Aquagenic pruritus may precede a diagnosis of polycythemia vera by several years or more.
- Close look at CBC in every case is important
- Even slight Hb increase in appropriate clinical situation should alert us for a diagnosis of polycythemia.
- Easy treatment is available for this disease which is not expensive.
- If we miss this diagnosis a patient may present with life threatening coronary heart disease or stokr
- Health check up can be useful to spot the diagnosis.
Jeevan Amrut Haematology Center News
With Dear Friend Dr Sudhakar Hase (MD Pulmonary Medicine and IDCCM ) joining in as an intensivist, we will be able to manage difficult and critical cases at Jeevan AMrut only, with our 4 beded sate-of-the-art ICU.
We have expanded Our Lab also with in house D-dimer, Procalcitonin, TSH, Iron studies etc for quick assessment of patients.
Jeevan Amrut Newsletter –
We will be meeting you at regular intervals on this virtual platform to discuss interesting hematology cases, clinical and laboratory updates and various news related to hematology through this newsletter.
Seeking your blessings.
Dr Manoj Toshniwal