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Clinical meet
 

Clinical meet

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    Clinical meet Clinical meet Presentation Transcript

    • Clinical meet Dr.H.B.Prasad Unit A case of PLHIV with Paraplegia Dr.Ajitkumar K.Jadhav JR (II) Date :- 2 nd April , 2011
    • HISTORY
      • 42 yr , male , Labourer , r/o Pune .
      • K/c/o PLHIV since last 1 ½ yrs on ART (ZL+ Efa )
      • CD4- 120/cmm
      • Chief complaints
        • Inability to walk - 2 months
        • weakness started in both lower limbs, associated with looseness , progressed to bed ridden state in last 15 days
        • Fever - on and off since 1 month
        • moderate grade , associated with night sweats , relieved with antipyretics
        • Easy fatigability and exertional dyspnoea – 1month
      • H/o Pulmonary Kochs 6 months back ,was still on AKT
      • No h/o
        • trauma to back
        • Similar episode in the past
        • Decreased sensations below the level of umbilicus
        • No bowel/bladder complaints
    • Examination
      • General Examination:
        • Avg. built ,poorly nourished
        • Ht -157 cm ,Wt – 40 kg ,BMI - 17
        • Conscious ,oriented
        • Febrile
        • Pulse - 102/min
        • BP - 110/70 mmHg
        • Pallor ++
        • Multiple matted lymph nodes in Cervical region ,1 cm in size
        • No oedema /icterus / cyanosis /clubbing
      • Systemic Examination :
        • CVS – S 1 S 2 heard well, Tachycardia
        • RS – Breath sounds were decreased in Rt. IAA with stony dull purcussion note ,with no adventious sounds
        • Rest lung fields were clear
        • P/A –Soft , Liver -palpable 3 cm , firm
        • Spleen - Palpable 5 cm , firm
        • No free fluid
        • CNS –
        • Higher functions and Cranial nerves were normal
        • Motor system
        • Nutrition – mild wasting was present
        • Tone –Normal in both Upper Limbs and decreased in both Lower Limbs
      • Power - Grade V in both upper limbs
      • Grade II in both lower limbs
      • Co-ordination – Normal in upper limbs
      • Could not be tested in lower limbs
      • Reflexes – DTR ++ in both upper limbs
      • +++ in both lower limbs
      • Superficial- lower abdominals were absent
      • Plantars were both extensors
      • Sensory system
      • Pain , Temp,Touch
      • Joint position & Vibration
      • Spine showed gibbus at the level of T-8 vertebra , Which was tender
      decreased below the level Umbilicus around T 12
      • Young male PLHIV on ART , with pulm kochs on AKT with anemia (likely AZT induced ) with chronic progressive paraplegia , with sensory involvement ,without bowel / bladder involvement and with lymphadenopathy and hepato-splenomegaly
      • Differentials
      • ?
      • Compressive myelopathy involving thoracic spine likely
        • Potts spine
        • TB myelitis
        • Disseminated hematological malignancy like Lymphoma
        • bacterial spinal abscess
    • Investigations
      • Hb- 3.7 gm %
      • MCV – 112 fl
      • TLC – 9700/ cmm
      • DLC – P 67 L 26 E 03 M 04 B 0
      • mild anisocytosis,normocytes 2 +, microcytes 1 + ,few macrocytes ,polychromasia 2 +, moderate hypochromia
      • Platelet count - 2.48 lac
      • Retic count -8 %
      • ESR- 57 mm at the end of 1 hr
      • Sr. Creatinine 1.7 mg %
      • Total bilirubin – 2.5 mg % , Direct 1.6 mg%
      • SGOT/SGPT – 45/39 (IU/L)
      • Total proteins – 5.4 gm %
    • Chest Xray
      • -Right sided loculated
      • Pleural effusion with
      • Underlying lung collapse
      • pleural thickening
          • USG abdomen
          • Hepatosplenomegaly with mild ascitis
          • Rt psoas abscess with echogenic mesentry
          • B/L enlarged echogenic kidneys
    • X ray Thoraco-lumbar spine
      • Showing compression collapse of D9-D10 vertebrae with anterior wedging in lateral film
    • MR Scan of dorsal spine reveals :-
      • Altered marrow signals appearing hypointense on T1 and hyperintense on STIR images are noted in D6 , D7 to D8 & 9 vertrbral bodies and posterior neural arches of D9 & D10 vertebrae. s/o infiltration and inflammation in vertebral bodies
      • Pre and bilateral paravertebral T2 hyperintense soft tissue component extending from D3 to D11 vertebral bodies ( abscesses ) with anterior epidural soft tissue seen from D9 to D10
      • Swollen cord with T2 hyperintense signals longitudinally along D8/10 vertebra suggestive of cord edema / infective myelitis.
      • Loculated thick walled right pleural effusion –? empyema.
      In view of reduction of intermediate disc spaces , associated pre & para spinal abscesses, possibility of multicentric tuberculosis is more likely, needs histopathological corelation
          • Patient was continued on
          • - ART (LS + Efa )
          • - AKT with addition of steroids,hematinics .
      • The question that remained unanswered was why did patient develop paraplegia despite being on AKT
      • ???
      • Meanwhile we received a PBS report from pathology dept stating that
      • RBC : mild anisocytosis , microcytes 1 + ,few macrocytes polychromasia 2 +, moderate hypochromia
      • TLC : 60000 /cmm
      • DLC :
      • Neutrophils :- 35 %
      • Monocytes :- 5 %
      • Lymphocytes – 5 %
      • Abnormal cells – 55% abnormal cells are large cells with High N:C ratio , folded nucleus and scanty , bluish cytoplasm. Few cells show prominent nucleoli. Suggestive of Lymphoid cells
      • Platelet : appear scattered and reduced.
      • Parasites : Not seen in present smear
      • Advice : Follow up with bone marrow aspiration .
      • Special stain report follows.
      • BM Aspiration
      • Hypercellular marrow with diffuse infiltration by large lymphoid cells of heterogenous morphology
      • Erythroid series : Megaloblastic erythropoiesis
      • Granulocyte series : relatively depressed
      • Myeloid / Erythroid ratio : 9:1
      • Differential counts :
      • Blast like cells - 70 % Basophils -00 %
      • Promyelocytes – 01% Eosinophils -01%
      • Myelocytes - 03% Lymphocytes- 04%
      • Metamyelocytes -02% nRBC : 10%
      • Band cells – 03% Plasma cells -03%
      • IMPRESSION :
      High grade Non –Hodgkin’s Lymphoma (? Burkitt’s Lymphoma )
    • Final Diagnosis
      • Patient of PLHIV with Pulmonary Koch’s(On ART and AKT ) with High grade NHL (? Burkitt’s Lymphoma ) with AZT induced anemia with compressive myelopathy most likely due to TB and Lymphoma
      • As patient was critically ill ,and non affording , immunophenotyping and cytogenetics studies was not possible
      • As a desperate measure we started him on chemotherapy ---Cyclophosphamide , Vincristin , Adriamycin and Prednisolone , as per Hemato -oncologist ‘s opinion
      • During the course of treatment pt developed altered sensorium, for that Lumbar puncture was tried but no fluid could be tapped .
      • Unfortunately we lost the pt during the course of illness
      • (Day 2 of chemotherapy)
    • Discussion
      • Spinal cord involvement in HIV patients
      • Myelopathy is seen ~20% of patients with AIDS, often as part of
      • HIV encephalopathy
      • CMV infection causes Progressive Lumbosacral Polyradiculopathy
      • HTLV-I-associated myelopathy (HAM) -Tropical spastic paraparesis
      • Neurosyphilis (Meningeal, Meningovascular , General paresis , Tabes dorsalis )
      • Infection with herpes simplex or varicella-zoster , EBV (Rapidly progressive symmetric lower limbs weakness)
      • TB
      • Lymphoma
      • Lymphomas in HIV
      • Lymphoma is a late manifestation of HIV infection, generally occurring in patients with CD4+ T cell counts <200/ uL
      • 90% are B cell in phenotype, and
      • half contain EBV DNA
      • Three main categories
      • Grade III or IV immunoblastic lymphoma :-
        • 60 % of all lymphomas ,
        • associated with pleural, pericardial, and/or peritoneal effusions in the absence of discrete nodal or extranodal masses,
        • HHV-8 DNA sequences have been found
      • Burkitt's lymphoma :- 20% cases , Small noncleaved cell lymphoma
      • Primary CNS lymphoma . :-
        • Mostly positive for EBV ,
        • generally presents with focal neurologic deficits, including cranial nerve findings, headaches, and/or seizures.
      • Burkitt’s lymphoma
      • 30%–40% of non-Hodgkin’s lymphoma   in HIV +  patients
      • Burkitt’s   lymphoma was estimated to be 1,000 times more common in HIV +  individuals than in the general population  
      • The diagnosis of Burkitt’s lymphoma in an HIV +  individual   often represents the first AIDS-defining criterion.
      • HIV is involved in lymphomagenesis   via
        • cytokine deregulation
        • chronic   antigenic stimulation
        • decreased immune surveillance
      • Lymphoma often involves lymph nodes, bone marrow, and extranodal   sites, most often in the abdomen , liver ,CNS and lungs
      • Burkitt,s lymphoma in HIV is very aggressive tumour with poor response to chemotherapy , difficult to achieve complete remission , and less proven response to Rituximab .  
      • Multicentric Castleman's disease –
      • This is an HHV-8 associated lymphoproliferative disorder , usually associated with generalized lymphadenopathy, hepatosplenomegaly, and systemic symptoms of fever, fatigue, and weight loss.
      • Treatment recommendations:
      • Systemic non-Hodgkin’s lymphoma
      • All patients with CD4 <100/mm 3 :  Low-dose chemotherapy
      • CD4 >100/mm 3 :  Consider standard-dose chemotherapy in selected patients.
      • Antibiotic prophylaxis  for P. carinii in all patients.
      • Meningeal prophylaxis  for those with small noncleaved histology, bone marrow involvement, paranasal sinus involvement, or epidural disease.
      • Antiviral therapy:
      • Combination antiviral therapy should be continued with chemotherapy
      • Zidovudine is not recommended due to risk of overlapping myelosuppression.
        • Rituximab, a monoclonal anti-CD20 antibody.
      • THANK YOU