Casepresentation
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-posterior cervical triangle -axillary Petechial Haemorrhages noticed on the gums, hard palate, tongue and forearms bilaterally Bruises noticed over the anterior aspect of both foreams Vitals: BP122/51 Pulse 68, regular, good volume RR 28bpm Temp 36.8 C
THROMBOCYTOPENIA
Normal spleen
Normal marrow
Abnormal Marrow Aplasia
Haem d/o
Excess destruction
Production defect
Immune Drugs
Idiopathic
Non-immune Prostehesis Sepsis/DIC Vasculitis
Case presentation
Mr K Ntombela, 44 year old gentleman Referred from CJM hosp, and presented to Grey’s Hospital on 6th
February 2003 with a bicytopenia Hb 2,2 and platelet count of 34. On arrival to CJM the patient was actively bleefuse him with 2 units of packed red cells and platelets, with no response
•U & E: Na 129 K2,3 Cl 101 HCo3 U 4,7 Cr 132
•INR 0,85
•PTT 20,1(p) 29,7(c)
•Bleeding time : prolonged
•Blood cultures: negative
•Malaria smear: negative
•Urine dipstix: trace blood, 1+leuco’s and 1+blood •BMA: inadequate spp •BMT: showed hypercellularity,all the haemopoeitic elements are seen. Good maturation noticed in the myeloid and erythroid series. Megakaryocytes are increased in number. Some have dysmorhic morphology with micro-megakaryocytes and hyperchromatic nuclei. There is no increase in reticulin fibrosis and no abnormal infiltrate. Granulomas not noticed.
Patient is currently being managed further at KEH.
Conclusion
Here we have a 44 year old gentleman who suddenly developed a bleeding diasthesis, following which relevant work-up confirmed an idiopathic thrombocytopenic purpura. As to the underlying predisposing factor, we felt that the most likely precipitant was his retroviral positive status, which brings us to the core of our discussion today: HIV and its effects it has on the haematological system.
tendencies. z No other bleeding diasthesis reported z No history of trauma, toxin ingestion, or any other haematological
disorders, including family hx z Describes one other similar episode last year March 2002. Managed
Neurological- painful paraesthesia’s soles of feet dizziness (non-vertiginous)
No other significant past medical or surgical history Medications- no previous ingestion of anti-inflammatory’s,
Peripheries
Nails-no koilonychia Bruises of varying sizes over arms and lower limbs No bony tenderness ? Kaposi’s sarcoma lesion over anterior tibial surface LHS
Findings suggestive of ITP
•RVD status – positive total lymphocytes 800 CD4 count not available yet
Management
Patient was initially commenced on haematinics (pregamal, Vit B12), with 2 units of packed red cells and 5 units of pooled platelets being transfused. Subsequent repeat bloods and the patients clinical condition remained unchanged (platelets dropped to 5), suggesting an immune process in place. On confirmation of the diagnosis of ITP via BMT, oral prednisone 60mg dly was initiated with once again no clinical and biochemical improvement.
Bone Marrow
Usually displays a decreased cellularity and myelodysplasia Decreased CD34 and Progenitor cells Increase cytokines TGF, TNF-alpha, IFN-gamma, IL-1 Drug induced Infections
Respiratory:
NAD (including no hilar lymphadenopathy)
Abdominal
No liver or spleen palpated No features of liver failure No abdominal lymphadenopathy PR: malaena
anticoagulants etc. Family history-nil Ethic origin-Zulu(KZN)
On examination
Generally Well looking No active bleeding Pallor ++ Not jaundiced Oral thrush noted Lymphadenopathy (<1cm)
Dr Asmal (haematologist) at ALH was contacted on 24/2 and it was decided in view of the marked thrombocytopenia which was not responding to oral steroids and the patients RVD positive status, a splenectomy be considered as a next step.
Cardiovascular
Normotensive Not in Failure, no features of infective endocarditis S1 and S2 present and normal
Short ESM at LPSB grade 2/6- probably in keeping with a a flow murmur
Red blood cells
Anaemia is the most common abnormality in HIV infection. There is a disproportionate decrease in reticulocytes. Usually a normocytic normochromic anaemia manifests. A positive coombs tests may be present in 18-100% of patients. Of interest,a persistently low haemoglobin is predictive of a poor outcome.
by a private GP who transfused 2 units of packed cells, with no history of further investigations or follow-up thereafter.
Systemic enquiry
CVS- Fatigue, dyspnoea grade II, No orthopnea or PND
Main complaint: z 4 day history of sudden onset epistaxis followed more recently by
haematemesis (dark red blood) z In addition reports bleeding from the gums with easy bruising
Bloods
Special investigations
FBC
31/1 06/2 14/2 16/2 22/2
HB
2,2 5,4
WCC
23,5 12.3
Platelets 34
9
Corr retics 4,95
RPI
1,9
3,4 6,0 8,3 7,3 7,0 11,3 13 3,0 5,0
Smear showed target red cells with assoc anisocytosis. Unfortunately no comment was made on platelets.
Splenomegaly
Normal marrow Liver disease
Tumour Congestive splenomegaly
Abnormal marrow Leukaemia Lymphoma Haem d/o
HAEMATOLOGY AND HIV
Haematological effects are common in HIV infection. The severity of its effects increases in advanced disease Haematological influences may be due to: HAART, opportunistic infections, malignancy or HIV infection itself. These may range from deviation in one cell line to a pancytopenia.
THROMBOCYTOPENIA
Normal spleen
Normal marrow
Abnormal Marrow Aplasia
Haem d/o
Excess destruction
Production defect
Immune Drugs
Idiopathic
Non-immune Prostehesis Sepsis/DIC Vasculitis
Case presentation
Mr K Ntombela, 44 year old gentleman Referred from CJM hosp, and presented to Grey’s Hospital on 6th
February 2003 with a bicytopenia Hb 2,2 and platelet count of 34. On arrival to CJM the patient was actively bleefuse him with 2 units of packed red cells and platelets, with no response
•U & E: Na 129 K2,3 Cl 101 HCo3 U 4,7 Cr 132
•INR 0,85
•PTT 20,1(p) 29,7(c)
•Bleeding time : prolonged
•Blood cultures: negative
•Malaria smear: negative
•Urine dipstix: trace blood, 1+leuco’s and 1+blood •BMA: inadequate spp •BMT: showed hypercellularity,all the haemopoeitic elements are seen. Good maturation noticed in the myeloid and erythroid series. Megakaryocytes are increased in number. Some have dysmorhic morphology with micro-megakaryocytes and hyperchromatic nuclei. There is no increase in reticulin fibrosis and no abnormal infiltrate. Granulomas not noticed.
Patient is currently being managed further at KEH.
Conclusion
Here we have a 44 year old gentleman who suddenly developed a bleeding diasthesis, following which relevant work-up confirmed an idiopathic thrombocytopenic purpura. As to the underlying predisposing factor, we felt that the most likely precipitant was his retroviral positive status, which brings us to the core of our discussion today: HIV and its effects it has on the haematological system.
tendencies. z No other bleeding diasthesis reported z No history of trauma, toxin ingestion, or any other haematological
disorders, including family hx z Describes one other similar episode last year March 2002. Managed
Neurological- painful paraesthesia’s soles of feet dizziness (non-vertiginous)
No other significant past medical or surgical history Medications- no previous ingestion of anti-inflammatory’s,
Peripheries
Nails-no koilonychia Bruises of varying sizes over arms and lower limbs No bony tenderness ? Kaposi’s sarcoma lesion over anterior tibial surface LHS
Findings suggestive of ITP
•RVD status – positive total lymphocytes 800 CD4 count not available yet
Management
Patient was initially commenced on haematinics (pregamal, Vit B12), with 2 units of packed red cells and 5 units of pooled platelets being transfused. Subsequent repeat bloods and the patients clinical condition remained unchanged (platelets dropped to 5), suggesting an immune process in place. On confirmation of the diagnosis of ITP via BMT, oral prednisone 60mg dly was initiated with once again no clinical and biochemical improvement.
Bone Marrow
Usually displays a decreased cellularity and myelodysplasia Decreased CD34 and Progenitor cells Increase cytokines TGF, TNF-alpha, IFN-gamma, IL-1 Drug induced Infections
Respiratory:
NAD (including no hilar lymphadenopathy)
Abdominal
No liver or spleen palpated No features of liver failure No abdominal lymphadenopathy PR: malaena
anticoagulants etc. Family history-nil Ethic origin-Zulu(KZN)
On examination
Generally Well looking No active bleeding Pallor ++ Not jaundiced Oral thrush noted Lymphadenopathy (<1cm)
Dr Asmal (haematologist) at ALH was contacted on 24/2 and it was decided in view of the marked thrombocytopenia which was not responding to oral steroids and the patients RVD positive status, a splenectomy be considered as a next step.
Cardiovascular
Normotensive Not in Failure, no features of infective endocarditis S1 and S2 present and normal
Short ESM at LPSB grade 2/6- probably in keeping with a a flow murmur
Red blood cells
Anaemia is the most common abnormality in HIV infection. There is a disproportionate decrease in reticulocytes. Usually a normocytic normochromic anaemia manifests. A positive coombs tests may be present in 18-100% of patients. Of interest,a persistently low haemoglobin is predictive of a poor outcome.
by a private GP who transfused 2 units of packed cells, with no history of further investigations or follow-up thereafter.
Systemic enquiry
CVS- Fatigue, dyspnoea grade II, No orthopnea or PND
Main complaint: z 4 day history of sudden onset epistaxis followed more recently by
haematemesis (dark red blood) z In addition reports bleeding from the gums with easy bruising
Bloods
Special investigations
FBC
31/1 06/2 14/2 16/2 22/2
HB
2,2 5,4
WCC
23,5 12.3
Platelets 34
9
Corr retics 4,95
RPI
1,9
3,4 6,0 8,3 7,3 7,0 11,3 13 3,0 5,0
Smear showed target red cells with assoc anisocytosis. Unfortunately no comment was made on platelets.
Splenomegaly
Normal marrow Liver disease
Tumour Congestive splenomegaly
Abnormal marrow Leukaemia Lymphoma Haem d/o
HAEMATOLOGY AND HIV
Haematological effects are common in HIV infection. The severity of its effects increases in advanced disease Haematological influences may be due to: HAART, opportunistic infections, malignancy or HIV infection itself. These may range from deviation in one cell line to a pancytopenia.