Physicians Awareness To Haematology (PATH) Newsletter

Dear friends, colleagues and seniors,

Wish you all had a great festive season and enjoyed your Diwali vacation. We at Jeevan Amrut  wish you all the very best for the coming year. I am glad to share that we have successfully performed 40 bone marrow transplants till date. The indications included acute leukemia, aplastic anaemia, lymphoma and multiple myeloma. The unit was closed for 8 months due to COVID 19 pandemic and happy to share that we have restarted our bone marrow transplant program. I hope you keep your faith in our institute and help us serve better. Today we will be discussing one very interesting case with an important massage of “Not ignoring thrombocytopenia”and I hope you enjoy it.

This young man was timely referred by Dr. M.A. Abbasi, Dr. Farooq Ghanchi and Dr Deepak Gore sir – team from Universal Hospital, Nanded and I am very glad to share this case snippet with you all.

Mr Mustak, from Wasmat, a 20 year old Male, presented with a history of fever, pain in the abdomen, vomiting and loose motions for 8 days. This was followed by reddish, small, patchy, skin lesions. His Platelet count was around 40k on repeated blood reports. Dengue and malaria was negative. In 2 days he patient developed deteriorating consciousness and altered sensorium and he was referred early for thrombocytopenia for super specialty consultation rather than getting treated as – viral fever (?)

On admission, his vitals were stable and there was a petechial and ecchymotic rash all over the body. CNS examination showed that the patient was drowsy due to sedation, moving all four limbs and was getting completely disoriented with aggressive behavior once out of sedation. Investigations revealed hb to be 7.1  gm% with reticulocyte count of 12 %( increased ). Platelet count was 36,000/cumm. Bleeding time was 9½ minutes and coagulation screen was normal. His LFT showed indirect bilirubin of 3.6 with 4.1 total bilirubin. The peripheral smear revealed more than 20 schistocytes, hypochromia and anisocytes. (Photo) The platelets were markedly reduced with presence of giant forms. KFT was normal but Urine output was low and examination showed urine albumin of 2+ and 60-70 RBCs per high power field. Other investigations were not contributory.

Peripheral blood (Wright Stain, 1000x) showing schistocytes, characteristic of microangiopathic hemolytic anemia.

Considering clinical picture of typical pentad of TTP with fever, thrombocytopenia, hemolysis, neurological and renal involvement he was diagnosed with Thrombotic thrombocytopenic perpura (TTP). The patient was started with FFP transfusion and 1 gm methylprednisolone IV X 2 days with aspirin and supportive measures. Not affordable for ADAMTS 13 level or Plasma exchange. He rapidly regained complete consciousness in the ward within 24 hours. No fresh petechiae developed after 2 days of admission. All skin lesions disappeared by 5 days. Over a period of 1 weeks, the patient showed progressive improvement in clinical and biochemical parameters.

At the time of discharge, his clinical examination was totally normal. BUN and serum creatinine were 15 mg% and 1.4 mg% respectively. Urine examination and haemogram were normal.
Follow up over a period of one months revealed no clinical or biochemical deterioration or recurrence. Peripheral smear showing very schistocytes. He is now on slow tapering doses of steroids.

Peripheral blood (Wright Stain, 1000x) – after recovery showing very few schistocytes,

Take home messages from this case:

  1. Even mild to moderate thrombocytopenia should also be investigated properly especially when not fitting in to the clinical context.
  2. Peripheral smear examination by expert eyes with a sound clinical backup still remains the gold standard.
  3. When used with fresh plasma, corticosteroids and anti-platelet agents, patients may show dramatic improvement.
  4. Plasmapheresis may not be needed if treated early and aggressively.
  5. This is a serious disease, which diagnosis and the early establishment of the therapy is capable of improving the prognosis, including the possibility of cure.

Haematology New from across the world –

Gene therapy in thalassemia major – Patients with transfusion-dependent beta-thalassemia who received betibeglogene autotemcel (Zynteglo, bluebird bio) achieved durable transfusion independence with near-normal hemoglobin levels.

https://www.healio.com/news/hematology-oncology/20211212/gene-therapy-potentially-curative-for-patients-with-transfusiondependent-betathalassemia

Jeevan Amrut Newsletter 

Happy to share with you all that ” Jeevan Amrut Haematology Center” is now NABH certified superspeciality center.

Thank you

This is something new we have started with the intention to meet 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. Its my humble request to share your thoughts or ideas regarding this initiative.

in 2022 – We are coming with monthly newsletter this year with interesting hematology case/news or new research paper.

Seeking your blessings.

Thank you

Dr Manoj Toshniwal – Contact No – 9225300842

dr.manojtwal@jeevanamrut.com

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