A case of Myelofibrosis presentation 3.pptx

A case of myelofibrosis

1. Welcome To Weekly Central Presentation

2. A 55-year-old Male with Anemia and Abdominal Lump Presented By Dr Qazi Sazib Ahamed FCPS Part II Trainee Medicine Unit-04 Shaheed Suhrawardy Medical College Hospital

3. PARTICULARS OF THE PATIENT Name : Romjan Pramanik Age : 55 years Sex : Male Religion : Islam Occupation : Farmer Present Address : Mirpur, Dhaka Permanent Address : Pabna Date of admission : 29.06.2025 Date of examination : 29.06.2025

4. PRESENTING COMPLAINTS Generalized weakness for 1 year Feeling of a lump in left upper abdomen for 1 year Headache, palpitation, breathlessness for 1 year

5. History Of Present Illness According to the statement of the patient he was reasonably well 1 year back. Since then he was suffering from generalized weakness. It was increasing gradually in intensity and duration day by day and also hampering his daily activities. There was no diurnal variation. He also complained of feeling of a lump in the left upper abdomen for same duration.

6. History Of Present Illness Cont. It was associated with a dragging dull aching pain which was mild to moderate in intensity, gradual in onset, located mostly in the left upper part of abdomen, no radiation, aggravated by taking deep inspiration, partially relieved by taking analgesic and on right lateral recumbent position. He also complained of headache, palpitation, breathlessness. On query he also

7. History Of Present Illness Cont. complained of anorexia and significant weight loss of 1 kg in last 1 year which was unintentional. There was no history of fever, night sweat, cough, blood mixed with cough or vomiting or stool, blurring of vision, loss of consciousness, limb weakness, joint pain, rash, oral ulcer, photosensitivity, diarrhoea , constipation, bleeding manifestation, itching.

8. History Of Present Illness Cont. He didn’t give any history of jaundice, foreign travel, IV drug abuse, drinking alcohol, extra marital unprotected sexual exposure. No history of contact with known TB patients. During the course of illness he got 10 units of blood transfusions in different hospitals.

9. History Of Present Illness Cont. With the above mentioned complaints he visited several government hospitals but his condition didn’t improve. So he was referred to Shaheed Suhrawardy Medical College Hospital through out patient department for further management.

10. PAST HISTORY History of chronic hepatitis B virus infection, the duration of which couldn’t be mentioned by the patient.

11. TREATMENT HISTORY He took several medications after consultation with several doctors which were - Calcium and Vitamin D, Omeprazole, Montelukast , Domperidone , Anti ischaemic drugs.

12. PERSONAL HISTORY Habituated with normal Bangladeshi food . No history of smoking, alcohol, substance misuse. He is a farmer from very beginning of his adulthood and uses organic/inorganic chemicals for farming.

13. SOCIAL HISTORY He came of a lower middle class family. His monthly income was 20,000 taka. But due to his illness currently unemployed. He lives in a brick build house. Drinks supplied water. Uses sanitary latrine.

14. FAMILY HISTORY 2nd issue of non consanguineous parents. Six members in his family. Has got two sons and two daughters. All of them are in good health. Father died due to geriatric illness.

15. ALLERGIC HISTORY He was not known to be allergic to any drugs or food. IMMUNIZATION HISTORY He was not immunized according to EPI schedule. He was not vaccinated against Hepatitis B virus. Two doses of Covid-19 vaccine was taken.

16. Travelling History No history of foreign travel or travelling to malaria, kala-azar endemic area.

17. General examination Appearance : Ill looking Body Build : Average Nutrition : Weight 55 kg, Height 170 cm, BMI 19 kg/m² Decubitus : Right lateral position Co- operation : Co- operative

18. General examination Cont. Pulse: 104 beats/min (Regular, Normal volume) Blood pressure: 110/70 mmHg (No postural drop) Temperature: 99⁰F Respiratory rate: 18 breaths/min Anemia: Present ( Moderate /++)

19. General examination Cont. Jaundice : Absent Cyanosis : Absent Clubbing : Absent Koilonychia : Absent Leukonychia : Absent Edema: Absent

20. General examination Cont. Dehydration: Absent Bony tenderness: Absent Pigmentation: Absent Lymph nodes: Not palpable Thyroid gland: Not enlarged

21. General examination Cont. Body hair: Normal Skin condition: Normal Spine: Normal Bed side urine for protein: Absent

22. Gastrointestinal System Mouth and pharynx: Lips, Teeth and gum, Oral mucous membrane, Tongue, Palate and movement of soft palate, Tonsils, Fauces – Normal Inspection: Shape of the abdomen – Asymetrically distended in left side Flanks - Normal

23. Gastrointestinal System Cont. Movement with respiration - Normal Umbilicus – Centrally placed, inverted Visible peristalsis and pulsation, Engorged veins – Absent Striae , Scar mark, Pigmentation, Swelling – Absent Cough impulse - Absent

24. Gastrointestinal System Cont. Groin, pubic hair and genitalia (with permission of the patient)- Normal Palpation: Local temperature – Normal Tenderness, Rigidity – Absent

25. Gastrointestinal System Cont. Liver – Enlarged, 5 cm from the right sub costal margin at right mid clavicular line, surface is smooth, margin sharp, firm in consistency, non tender, moves with respiration, upper border of liver dullness is in the right fifth intercostal space, no hepatic bruit.

26. Gastrointestinal System Cont. Spleen: Palpable and hugely enlarged, 20 cm from left sub costal margin along left anterior axillary line towards the right iliac fossa, surface smooth, splenic notch present, firm in consistency, moves with respiration, non tender, finger insinuation could not be possible, percussion note dull, splenic rub absent.

27. Gastrointestinal System Cont. Kidneys – Are not ballotable Gallbladder – Not palpable Para-aortic lymph nodes – Not palpable Hernial orifice - Intact Testis (with permission of the patient) - Normal Digital rectal examination – Normal

28. Gastrointestinal System Cont. Percussion: Shifting dullness – Absent Percussion note – Tympanitic Auscultation: Bowel sound – Present Renal bruit – Absent

29. Cardiovascular System Inspection: Any deformity of the chest – Absent Visible cardiac impulse and other impulses – Absent Any scar mark – Absent

30. Cardiovascular System Cont. Palpation: Apex beat – Left fifth intercostal space, lateral to the left mid clavicular line, 10cm from mid sternal line, normal in nature. Thrill – There is a systolic thrill present in left lower para-sternal area.

31. Cardiovascular System Cont. Left parasternal heave, Palpable P2, Epigastric pulsation – Absent Auscultation: 1st and 2nd heart sounds – Normal and audible in all four areas . Systolic flow murmur in left lower para sternal area.

32. Respiratory System Inspection: Shape of the chest, Movement of the chest, Intercostal space – Normal Deformity, Drooping of the shoulder – Absent Visible impulse and engorged vein, Scar mark – Absent

33. Respiratory System Cont. Palpation: Position of trachea – Centrally placed Chest expansion – Normal Chest movement – Symmetrical Local rib tenderness – Absent

34. Respiratory System Cont. Apex beat – Left fifth intercostal space, lateral to the left mid clavicular line, 10cm from mid sternal line, normal in nature. Percussion : Percussion note – R esonant bilaterally .

35. Respiratory System Cont. Auscultation: Breath sound – Vesicular bilaterally. Vocal resonance – Normal on both sides. Added sound – Absent.

36. Nervous System Higher Psychic Functions (HPF): Normal Cranial nerves: Intact Fundoscopy : Normal Signs of meningeal irritation: Absent

37. Nervous System Cont. Motor functions: Bulk of the muscle – Normal Tone of the muscle – Normal Power of the muscle – Normal (5 out of 5 in all four limbs) Involuntary movement – Absent

38. Nervous System Cont. Coordination – Normal Gait and posture – Normal Superficial reflexes – Present (Abdominal reflex , Corneal reflex , Palatal reflex , Cremasteric reflex ) Deep reflexes - Side Biceps Triceps Supinator Knee Ankle Right Present Present Present Present Present Left Present Present Present Present Present

39. Nervous System Cont. Planter – Flexor bilaterally Sensory functions : Normal (Pain, Touch, Temperature, Position sense, Sense of vibration, Tactile localization, Tactile discrimination, Recognition of size and shape, weight and form of object) Romberg’s sign – Absent

40. Locomotor System Inspection: Swelling, local muscle wasting, any deformity, Redness, Skin change – Absent Palpation: Temperature – Normal Tenderness – Absent

41. Locomotor System Cont. Movement – Both active and passive movement in all joints are normal Spine – Normal SI Joint – Normal Nerve root compression – Absent

42. Salient Feature Mr Romjan Pramanik , 55 year, male, muslim , farmer, normotensive, non-diabetic, non-asthmatic, non smoker, hailing from Mirpur , Dhaka, admitted in this hospital through out patient department with the complaints of generalized weakness, feeling of a lump in left upper abdomen with occational pain, headache, palpitation, breathlessness

43. Salient Feature Cont. for one year. The pain was dragging dull aching in nature, mild to moderate in intensity, gradual in onset, located mostly in the left upper part of abdomen, no radiation, aggravated by taking deep inspiration, partially relieved by taking analgesic and on right lateral recumbent position. He also complained of anorexia and significant weight loss of 10 kg in last 1 year

44. Salient Feature Cont. which was unintentional. There was no history of fever, night sweat, cough , haemoptysis , haematemesis , melena, blurring of vision, loss of consciousness, limb weakness, joint pain, rash, oral ulcer, photosensitivity, diarrhoea , constipation, bleeding manifestation, jaundice, foreign travel, IV drug abuse, drinking alcohol, extra marital unprotected sexual

45. Salient Feature Cont. e xposure . No history of contact with known TB patients. During the course of illness he got 10 units of blood transfusions in different hospitals. He is a known case of chronic hepatitis B virus infection but the duration couldn’t be mentioned by the patient. There is no history of such type of illness in his family.

46. Salient Feature Cont. On general examination he was ill-looking, moderately anemic, tachycardia was present, rest of the findings were normal. On systemic examination, there was hepatomegaly (15cm) and massive splenomegaly (20cm ),apex beat was shifted, systolic thrill was present in left lower para-sternal area and also diastolic

47. m urmur in the same area. Rest of the findings of systemic examination revealed no abnormality.

48. Problem List

49. Provisional Diagnosis Chronic myeloid leukemia with Chronic Hepatitis B virus infection.

50. Differential Diagnosis Lymphoma Myelofibrosis Chronic malaria Disseminated Kala- azar CLD due to Chronic Hepatitis B virus infection with Chronic Hepatitis B virus infection

51. Investigations

52. CBC Test Name 3/7/25 29/6/25 6/5/25 8/5/24 Hb 7.0 g/dl 7.0 g/dl 8.6 g/dl 6.8 g/dl Total WBC 6440/ cmm 6540/ cumm 7100/ cumm 6260/ cumm Neutrophil 70% 68% 63% 71% Lymphocyte 20% 23% 29% 20% Monocyte 3% 05% 06% 6% Eosinophil 4% 04% 02% 3% Basophil 0% 00% 00% 0% Myelocytes 3% 7.6% Not seen Not seen RBC 2.35 mn / ul 2.23 mn / ul 2.41 mn / ul 2.32 mn / ul Platelet 2,40,000/ cmm 2,20,000/ cumm 2,77,000/ cumm 2,25,000/ cumm

53. Red Cell Indices Test Name 3/7/25 29/6/25 6/5/25 8/5/24 MCV 97.4 fL 96.9 fL 75.5 fL 94 fL MCH 29.8 pg 31.4 pg 36.6 pg 29.3 pg MCHC (32-36 g/dl) 30.6 g/dl 32.4 g/dl 47.2 g/dl 31.2 g/dl RDW-SD (39-46 fL ) 72.1 fL 72.2 fL 66 fL Not seen RDW-CV (11.6-14%) 20.7% 21.2% 23.6% 23.2%

54. PBF (3/7/25) RBC Shows Anisopoikilocytosis with hypochromasia . Significant number of polychromatic cells, tear drop cells and few nRBCs are seen. WBC Mostly mature with few myelocytes are seen. Platelet Adequate. Comment Leukoerythroblastic anemia.

55. Test Name Results Reticulocyte % 2.50% LDH 413 U/L Coombs test (Direct/Indirect) Negative Osmotic Fragility Test Normal Uric acid 7.9 mg/dl Iron 104 ug /dl TIBC 296 ug /dl Ferritin 392.3 ng /ml Hb electrophoresis Hb A 93.3% Hb F 4.6% Hb A2 2.1% Comment: Elevated Hb F.

56. Test Name Result Fasting Plasma glucose 5.40 mmol /l 2 hrs after 75 gm glucose 9.10 mmol /l HbA1c 7.0% Bilirubin 1.58 mg/dl (Total) 0.51 mg/dl (Direct) 1.07 mg/dl (Indirect) Albumin 4.2 g/dl SGPT 20 U/L Creatinine 1.13 mg/dl Na 139 mmol /l K 3.8 mmol /l Cl 105 mmol /l

57. Urine R/E Color Straw Appearance Normal PH 6.00 Albumin NIL Sugar NIL Epithelial cell 1-2 / HPF RBC NIL / HPF Pus Cell 2-4 / HPF Cast Absent / HPF

58. HBsAg : Positive Anti HCV : Negative HBeAg : Negative Anti HBe : Positive HBV DNA : Not detected

59. TSH : 3.36 uIU /ml ANA : Negative ICT for malaria : Negative ICT for Kala- azar : Negative

60. CEA : 1.0 ng /ml CA-19.9: 6.67 U/ml AFP : 32.76 ng /ml PSA : 2.5 ng /ml

61. Lipid Profile: Total cholesterol : 231 mg/dl HDL : 103 mg/dl LDL : 116 mg/dl TG : 61 mg/dl

62. Chest X-ray: Normal

63. ECG: Within Normal Limit

64. ECHO : Normal

65. USG Of Whole Abdomen: Liver is enlarged in size ( 15.8 cm ). Echotexture of liver parenchyma is homogenous with no focal abnormality. Spleen is hugely enlarged in size ( 22.1 cm in length) with uniform echotexture . Comment: 1. Hepatomegaly 2. Huge splenomegaly.

66. Endoscopy: Erosive Gastritis

67. Colonoscopy: Normal

68. Fibroscan : CAP:118dB/min E:9.6kPa F3 Fibrosis

69. Philadelphia Chromosome Analysis: Not detected

70. Bone Marrow Study: No marrow particle is seen. Advice: Trephine biopsy.

71. Histopathology of Trephine biopsy : Gross description: A core of bony tissue having a length of 1.4cm. Embedded as such after short decalcification.

72. Microscopic Appearance: Sections show a core of bony tissue. The marrow is cellular with normal M/E ratio. Erythropoiesis is depressed. Granulopoiesis is active with usual maturation. Megakaryocytes are present in usual number.

73. No reactive fibrosis , anaplastic cells or evidence of lymphoproliferation is seen.

74. Haematology Consultation

75. Reticulin stain reveals Grade 1 Fibrosis.

76. Final Diagnosis Myelofibrosis with HBeAg negative Chronic HBV infection with Diabetes Mellitus with Dyslipidemia.

77. Treatment Diet : Normal Maintain adequate hydration and nutrition Tab Hydroxyurea (500mg) – 1+0+0 Tab Folic acid (5mg) – 1+0+0 RCC transfusion Tab Linagliptin+Empagliflozin (5/10mg) – 1+0+0 Tab Atorvastatin (10mg) – 0+0+1

78. Plan MPN ( Myeloproliferative Neoplasm) reflex panel test from peripheral blood/bone marrow. Follow up after 14 days with repeat CBC.

79. Acknowledgement Department of Haematology ( ShSMC ). Department of Hepatology ( ShSMC ).